Bovine Spongiform Encephalopathy; MRR

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93

2012-01-05T11:45:00.000-08:00

Subject: Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Date: August 24, 2005 at 2:47 pm PST August 24, 2005

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Greetings APHIS ET AL,

My name is Terry S. Singeltary Sr.

I would kindly like to comment on [Docket No. 05-004-1] RIN 0579-AB93 ;

PROPOSED RULES

Exportation and importation of animals and animal products:

Whole cuts of boneless beef from- Japan,

48494-48500 [05-16422]

[Federal Register: August 18, 2005 (Volume 70, Number 159)]
[Proposed Rules]
[Page 48494-48500]
From the Federal Register Online via GPO Access [wais.access.gpo.gov]
[DOCID:fr18au05-7]

========================================================================

Proposed Rules

Federal Register

________________________________________________________________________

This section of the FEDERAL REGISTER contains notices to the public of
the proposed issuance of rules and regulations. The purpose of these
notices is to give interested persons an opportunity to participate in
the rule making prior to the adoption of the final rules.

========================================================================

[[Page 48494]]

DEPARTMENT OF AGRICULTURE
Animal and Plant Health Inspection Service
9 CFR Part 94
[Docket No. 05-004-1]
RIN 0579-AB93

Importation of Whole Cuts of Boneless Beef from Japan
AGENCY: Animal and Plant Health Inspection Service, USDA.
ACTION: Proposed rule.

-----------------------------------------------------------------------

SUMMARY: We are proposing to amend the regulations governing the
importation of meat and other edible animal products by allowing, under
certain conditions, the importation of whole cuts of boneless beef from
Japan. We are proposing this action in response to a request from the
Government of Japan and after conducting an analysis of the risk that
indicates that such beef can be safely imported from Japan under the
conditions described in this proposal.

DATES: We will consider all comments that we receive on or before
September 19, 2005.

ADDRESSES: You may submit comments by any of the following methods:

EDOCKET: Go to http://www.epa.gov/feddocket to submit or

snip...

BSE infectivity has never been demonstrated in the muscle tissue of
cattle experimentally or naturally infected with BSE at any stage of
the disease. Studies performed using TSEs other than BSE in non-bovine
animals have detected prions in muscle tissue. However, the
international scientific community largely considers that these studies
cannot be directly extrapolated to BSE in cattle because of the
significant interactions between the host species and the prion strain
involved.

Pathogenesis studies of naturally and experimentally infected
cattle have not detected BSE infectivity in blood. However,
transmission of BSE was demonstrated in sheep that received a
transfusion of a large volume of blood drawn from other sheep that were
experimentally infected with the BSE agent. The United Kingdom's
Department for Environment, Food and Rural Affairs' Spongiform
Encephalopathy Advisory Committee (SEAC) and the European Commission's
Scientific Steering Committee (SSC), which are scientific advisory
committees, evaluated the implication of this finding in relation to
food safety.\5\ The SEAC concluded that the finding did not represent
grounds for recommending any changes to the current control measures
for BSE. The SSC determined that the research results do not support
the hypothesis that bovine blood or muscle meat constitute a risk to
human health.\6\

snip...

BSE Risk Factors for Whole Cuts of Boneless Beef

The most significant risk management strategy for ensuring the
safety of whole cuts of boneless beef is the prevention of cross-
contamination of the beef with SRMs during stunning and slaughter of
the animal. Control measures that prevent contamination of such beef
involve the establishment of procedures for the removal of SRMs,
prohibitions on air-injection stunning and pithing, and splitting of
carcasses. These potential pathways for contamination and the control
measures that prevent contamination are described in detail in the risk
analysis for this rulemaking.

SRM Removal. Research has demonstrated that SRMs from infected
cattle may contain BSE infectivity. Because infectivity has not been
demonstrated in muscle tissue, the most important mitigation measure
for whole cuts of boneless beef is the careful removal and segregation
of SRMs. Removal of SRMs in a manner that avoids contamination of the
beef with SRMs minimizes the risk of exposure to materials that have
been demonstrated to contain the BSE agent in cattle.

snip...

Variant Creutzfeldt-Jakob disease (vCJD), a chronic and fatal
neurodegenerative disease of humans, has been linked since 1996 through
epidemiological, neuropathological, and experimental data to exposure
to the BSE agent, most likely through consumption of cattle products
contaminated with the agent before BSE control measures were in place.
To date, approximately 170 probable and confirmed cases of vCJD have
been identified worldwide. The majority of these cases have either been
identified in the United Kingdom or were linked to exposure that
occurred in the United Kingdom, and all cases have been linked to
exposure in countries with native cases of BSE. Some studies estimate
that more than 1 million cattle may have been infected with BSE
throughout the epidemic in the United Kingdom. This number of infected
cattle could have introduced a significant amount of infectivity into
the human food supply. Yet, the low number of cases of vCJD identified
to date indicates that there is a substantial species barrier that
protects humans from widespread illness due to exposure to the BSE
agent.

snip...

International Guidelines on BSE

International guidelines for trade in animal and animal products
are developed by the World Organization for Animal Health (formerly
known as the Office International des Epizooties (OIE)), which is
recognized by the World Trade Organization (WTO) as the international
organization responsible for the development of standards, guidelines,
and recommendations with respect to animal health and zoonoses
(diseases that are transmissible from animals to humans). The OIE
guidelines for trade in terrestrial animals (mammals, birds, and bees)
are detailed in the Terrestrial Animal Health Code (available on the
internet at http://www.oie.int). The guidelines on BSE are contained in
Chapter 2.3.13 of the Code and supplemented by Appendix 3.8.4 of the
Code.

snip...end

http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/05-16422.htm

http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/pdf/05-16422.pdf

Greetings again APHIS ET AL,

THIS is not correct. IN fact, there are several factors i would like to kindly address. Muscle tissue has recently been detected with PrPSc in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve) of the 11th BSE cow in Japan (Yoshifumi Iwamaru et al). also recently, Aguzzi et al Letter to the Editor Vet Pathol 42:107-108 (2005), Prusiner et al CDI test is another example of detection of the TSE agent in muscle in sCJD, Herbert Budka et al CJD and inclusion body myositis: Abundant Disease-Associated Prion Protein in Muscle, and older studies from Watson Meldrum et al Scrapie agent in muscle - Pattison I A (1990), references as follow ;

PrPSc distribution of a natural case of bovine spongiform encephalopathy

Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori-
kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa
Priori Disease Research Center, National Institute of Animal Health, 3-1-5
Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp

Abstract

Bovine spongiform encephalopathy (BSE) is a disease of cattle that causes
progressive neurodegeneration of the central nervous system. Infectivity
of BSE agent is accompanied with an abnormal isoform of prion protein
(PrPSc).

The specified risk materials (SRM) are tissues potentially carrying BSE
infectivity. The following tissues are designated as SRM in Japan: the
skull including the brain and eyes but excluding the glossa and the masse-
ter muscle, the vertebral column excluding the vertebrae of the tail, spinal
cord, distal illeum. For a risk management step, the use of SRM in both
animal feed or human food has been prohibited. However, detailed
PrPSc distribution remains obscure in BSE cattle and it has caused con-
troversies about definitions of SRM. Therefore we have examined PrPSc
distribution in a BSE cattle by Western blotting to reassess definitions of
SRM.

The 11th BSE case in Japan was detected in fallen stock surveillance.
The carcass was stocked in the refrigerator. For the detection of PrPSc,
200 mg of tissue samples were homogenized. Following collagenase
treatment, samples were digested with proteinase K. After digestion,
PrPSc was precipitated by sodium phosphotungstate (PTA). The pellets
were subjected to Western blotting using the standard procedure.
Anti-prion protein monoclonal antibody (mAb) T2 conjugated horseradish
peroxidase was used for the detection of PrPSc.

PrPSc was detected in brain, spinal cord, dorsal root ganglia, trigeminal
ganglia, sublingual ganglion, retina. In addition, PrPSc was also detected
in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve).
Our results suggest that the currently accepted definitions of SRM in
BSE cattle may need to be reexamined. ...

179
T. Kitamoto (Ed.)
PRIONS
Food and Drug Safety

================

ALSO from the International Symposium of Prion Diseases held in Sendai, October 31, to
November 2, 2004;

Bovine spongiform encephalopathy (BSE) in Japan

snip...

"Furthermore, current studies into transmission of cases of BSE that are
atypical or that develop in young cattle are expected to amplify the BSE
prion"

NO. Date conf. Farm Birth place and Date Age at diagnosis

8. 2003.10.6. Fukushima Tochigi 2001.10.13. 23

9. 2003.11.4. Hiroshima Hyogo 2002.1.13. 21

Test results

# 8b, 9c cows Elisa Positive, WB Positive, IHC negative, histopathology
negative

b = atypical BSE case

c = case of BSE in a young animal

b,c, No PrPSc on IHC, and no spongiform change on histology

International Symposium of Prion Diseases held in Sendai, October 31, to
November 2, 2004.

The hardback book title is 'PRIONS' Food and Drug Safety
T. Kitamoto (Ed.)

Tetsuyuki Kitamoto
Professor and Chairman
Department of Prion Research
Tohoku University School of Medicine
2-1 SeiryoAoba-ku, Sendai 980-8575, JAPAN
TEL +81-22-717-8147 FAX +81-22-717-8148
e-mail; kitamoto@mail.tains.tohoku.ac.jp

Symposium Secretariat
Kyomi Sasaki
TEL +81-22-717-8233 FAX +81-22-717-7656
e-mail: kvomi-sasaki@mail.tains.tohoku.ac.ip

================================

107
Vet Pathol 42:107–108 (2005)
Letters to the Editor

Editor:

Absence of evidence is not always evidence of absence.
In the article ‘‘Failure to detect prion protein (PrPres) by
immunohistochemistry in striated muscle tissues of animals
experimentally inoculated with agents of transmissible spongiform
encephalopathy,’’ recently published in Veterinary
Pathology (41:78–81, 2004), PrPres was not detected in striated
muscle of experimentally infected elk, cattle, sheep, and
raccoons by immunohistochemistry (IHC). Negative IHC,
however, does not exclude the presence of PrPSc. For example,
PrPres was detected in skeletal muscle in 8 of 32
humans with the prion disease, sporadic Creutzfeldt-Jakob
disease (CJD), using sodium phosphotungstic acid (NaPTA)
precipitation and western blot.1 The NaPTA precipitation,
described by Wadsworth et al.,3 concentrates the abnormal
isoform of the prion, PrPres, from a large tissue homogenate
volume before western blotting. This technique has increased
the sensitivity of the western blot up to three orders
of magnitude and could be included in assays to detect
PrPres. Extremely conspicuous deposits of PrPres in muscle
were detected by IHC in a recent case report of an individual
with inclusion body myositis and CJD.2 Here, PrPres was
detected in the muscle by immunoblotting, IHC, and paraf-
fin-embedded tissue blot. We would therefore caution that,
in addition to IHC, highly sensitive biochemical assays and
bioassays of muscle are needed to assess the presence or
absence of prions from muscle in experimental and natural
TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland

References

1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812–1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121–125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171–180,
2001

===================================

Corinna Kaarlela, News Director
Source: Jennifer O'Brien
jobrien@pubaff.ucsf.edu

415-476-2557

14 February 2005

Diagnosis of prions in patients should utilize novel strategy, team says
A technique for detecting prions in tissue, developed in recent years by
UCSF scientists, is significantly more sensitive than the diagnostic
procedures currently used to detect the lethal particles in samples of
brain tissue from patients, according to a study performed by a UCSF team.
The finding indicates that the diagnostic technique, known as the
conformation-dependent immunoassay (CDI), should be established as the
standard approach for brain biopsies of patients suspected of having the
disease, they say. The team is exploring whether the CDI might be
adapted to detect prions in blood and muscle.

The finding suggests that reliance on the current methods for detecting
prions in human brain tissue -- microscopic examination of tissue for
the telltale vacuoles that form in brain cells and immunohistochemistry
(IHC), which involves detecting prions in brain sections using prion
protein-specific antibodies -- may have led to an under diagnosis of the
disease in patients in recent years, they say. (A definitive diagnosis
of the disease in humans is made only on autopsy, when a
neuropathologist can analyze multiple brain regions for vacuoles and
evidence of prions by IHC, and it is estimated that only 50 percent of
human cases are autopsied, in part because many pathologists do not want
to risk infection during the autopsy.)

In the study, the team compared the ability of the CDI and the two
traditional diagnostic techniques to detect prions in various brain
samples from 28 patients diagnosed on autopsy as having one of several
human forms of the disease -- sporadic, familial or iatrogenic
Creutzfeldt-Jakob disease (CJD). While the CDI detected the biochemical
signal for prions in 100 percent of the samples studied, the traditional
tests failed to detect the prion in a high proportion of cases. For
example, in an experiment that focused on 18 brain regions from eight
patients with sporadic CJD, the CDI detected prions in 100 percent of
the samples, while IHC detected them in 22 percent and routine tissue
examination in 17 percent.

"In about 80 percent of the different brain regions examined, prions
were not consistently detected by either IHC or routine histology that
measure vacuolation. In contrast, the CDI was always positive in all
regions of the brain," says the lead author of the study, Jiri Safar,
MD, associate adjunct professor of neurology and a member of the UCSF
Institute for Neurodegenerative Diseases, which is directed by senior
author Stanley B. Prusiner, MD, UCSF professor of neurology and
biochemistry.

"These findings indicate that histology and immunohistochemistry should
no longer be used to rule out prion disease in single-site biopsy
samples," says Safar. "The superior performance of the CDI in diagnosing
prion disease suggests that the CDI be used in future diagnostic
evaluations of prion disease, particularly for single-site brain
biopsies during life"

"If the traditional techniques are used at autopsy, they must be applied
to many cortical and subcortical samples," says co-author Stephen J.
DeArmond, MD, PhD, UCSF professor of neuropathology.
Moreover, while the study examined the efficacy of the CDI in comparison
to the two techniques routinely used by neuropathologists to detect
prions in human brain tissue, previous studies at UCSF indicate that the
CDI is also significantly more sensitive than Western blot analysis, the
technology used with IHC to detect prions in brain tissue from cattle
suspected of having bovine spongiform encephalopathy (BSE). That IHC and
Western blot analysis are relatively insensitive methods, the
researchers say, supports their ongoing assertion that the CDI should
also be used to evaluate the brain tissue of cattle.

"The studies reported here are likely to change profoundly the approach
to the diagnosis of prion disease in both humans and livestock," says Safar.
More broadly, the scientists say, the high sensitivity of the CDI
suggests that CDI-like tests could also prove useful for diagnosing
other neurodegenerative diseases, such as Alzheimer's disease,
Parkinsons's disease and fronto-temporal dementias, all of which, like
prion diseases, involve various forms of protein misprocessing. These
diseases currently are diagnosed by neuropathological analysis and
immunohistochemistry.

"Whether immunohistochemistry underestimates the incidence of one or
more of these common neurodegenerative diseases is unknown, but the CDI
could shed light on these diseases," says co-author Bruce Miller, MD,
UCSF A.W. and Mary Margaret Clausen Distinguished Professor of Neurology
and director of the UCSF Memory and Aging Center.

The finding will be printed on-line and in print on March 1, 2005 in
Proceedings of the National Academy of Sciences.

The study brings into high relief the different detection strategies of
immunohistochemistry and the CDI, both of which involve revealing the
presence of prions, known as PrPsc, by applying antibodies to brain tissue.
Standard immunohistochemistry, developed in the DeArmond lab 20 years
ago, involves using an enzyme known as a protease, or a combination of
harsh acid and high temperature treatment, to destroy normal prion
protein (PrPC), which is ubiquitous in brain tissue. Once this occurs,
scientists apply fluorescently lit antibodies that react with residues
of the relatively resistant abnormal prion protein (PrPSc), thereby
highlighting it.

The limitation of this technique is that scientists have since learned
that there is a large part of the abnormal prion protein that is
protease sensitive, and that portion escapes detection by the standard
technique. Thus, this traditional method underestimates the level of
PrPSc in tissue.

The CDI addresses this limitation by revealing the region of PrPSc that
is exposed in the normal PrPC but is buried in infectious PrPSc, using
high affinity, newly generated antibodies that identify PrPSc through
the distinct shape of the molecule, independent of proteolytic
treatments. This makes it possible to detect potentially large
concentrations of protease sensitive PrPSc molecules.

Detractors would say that it is not necessary to detect the minute level
of infectious agent that the CDI is capable of revealing, as it would be
unlikely to be lethal, says Safar. But Prusiner and his colleagues
maintain that any risk is too great when it comes to having prions in
the food supply. In addition, because even low levels of prions are
extremely resistant to inactivation, they may contaminate the
environment for many years.

Prusiner won the 1997 Nobel Prize in Physiology or Medicine for
discovering that a class of neurodegenerative diseases known as
spongiform encephalopathies was caused by prions. Prion diseases develop
in humans, cattle, sheep, deer, elk and mink.

The CDI was developed by members of the Prusiner lab. The CDI
methodology has been licensed to InPro Biotechnology, Inc.
Prusiner, Safar, DeArmond and other members of the Institute for
Neurodegenerative Diseases are scientific advisors to, or own stock in,
InPro.

Other co-authors of the study were Michael D. Geschwind, Camille
Deering, Svetlana Didorenko, Mamta Sattavat, Henry Sanchesz, Ana Serban,
Kurt Giles, of UCSF, and Martin Vey, of Behring, Marburg, Germany, and
Henry Baron, of Behring, Paris.

The study was funded by the National Institutes of Health, the John
Douglas French Foundation for Alzheimer's research, the McBean
Foundation, the State of California, Alzheimer's Disease Research Center
of California and the RR00079 General Clinical Research Center.

The UCSF Institute for Neurodegenerative Diseases:

http://ind.medschool.ucsf.edu/.

FURTHER COMPARISON OF THE CDI TO THE STANDARD DIAGNOSTIC PROCEDURES,
PROVIDED BY STEPHEN J. DEARMOND, MD, PHD, UCSF PROFESSORS OF NEUROPATHOLOGY:

Explanation as to why the CDI is more sensitive than Western blot
analysis: Studies at UCSF during development of the CDI showed that CDI
could detect prions in brain homogenates at levels that fail to produce
disease in animals (bioassay for prions). Therefore, the CDI is more
sensitive than the bioassay method, which was considered to be the most
sensitive technique for detecting prions. In contrast, Western blot
analysis for prions is significantly less sensitive than the bioassay
and is, therefore, significantly less sensitive than the CDI. Currently,
the USDA uses a combination of Western blot analysis of brainstem
homogenates and immunohistochemistry of the medulla to test cattle
suspected of having bovine spongiform encephalopathy ("mad cow
disease"). The relative insensitivity of IHC and Western blot analysis,
says DeArmond, supports the UCSF scientists' ongoing assertion that the
CDI should also be used to evaluate the brain tissue of cattle.
DeArmond cites additional evidence about Western blot analysis from a
World Health Organization (WHO) study group, which compared the CDI
method with Western blots for detection of prions in sporadic and
variant CJD brains. Based on the smallest amount of prions that could
detected by the two techniques, they found that the CDI was from 1000-
to 100,000-fold more sensitive than Western blot analysis performed in
six different research laboratories (Minor et al. Standards for the
assay of Creutzfeldt-Jakob disease specimens. J. Gen. Virol. 85:
1777-1784, 2004).

Explanation as to why IHC for prions is less sensitive than the CDI: IHC
is routinely performed on formalin-fixed, paraffin-embedded samples of
brain. Formalin fixation markedly decreases the ability of antibodies to
bind to proteins in general, which greatly weakens the IHC signal for
prions (PrPSc). In contrast, homogenates for the CDI are not treated
with reagents that decrease prion antigenicity. Moreover, to concentrate
the PrPSc for measurement by the CDI, the homogenates are exposed to
phosphotungstic acid, which selectively precipitates both
protease-sensitive and protease-resistant PrPSc that comprise prions,
but not the normal prion protein conformer found in uninfected animals,
PrPC. This step results in a higher concentration of PrPSc for detection
by the CDI. Because the PrPSc was not exposed to proteases, the CDI
measures all forms of abnormally folded PrPSc molecules.
Protease-sensitive PrPSc can account for 50 percent of the total PrPSc.
For Western analysis, homogenates of brain are treated with protease to
eliminate PrPC; however, this step also eliminates protease-sensitive
PrPSc leaving only protease-resistant PrPSc for Western blot detection
and decreasing the PrPSc signal at least in half.

###

http://pub.ucsf.edu/newsservices/releases/200502147/

PNAS | March 1, 2005 | vol. 102 | no. 9 | 3501-3506

NEUROSCIENCE

Diagnosis of human prion disease

Jiri G. Safar *, , Michael D. Geschwind , , Camille Deering
*, Svetlana Didorenko *, Mamta Sattavat ¶, Henry Sanchez ¶,
Ana Serban * , Martin Vey ||, Henry Baron **, Kurt Giles *,
, Bruce L. Miller , , Stephen J. DeArmond * , ¶ and Stanley
B. Prusiner *, , ,

*Institute for Neurodegenerative Diseases, Memory and Aging
Center, and Departments of Neurology, ¶Pathology, and
Biochemistry and Biophysics, University of California, San
Francisco, CA 94143; ||ZLB Behring, 35041 Marburg, Germany;
and **ZLB Behring, 75601 Paris, France
Contributed by Stanley B. Prusiner, December 22, 2004

Abstract

With the discovery of the prion protein (PrP),
immunodiagnostic procedures were applied to diagnose
Creutzfeldt–Jakob disease (CJD). Before development of the
conformation-dependent immunoassay (CDI), all immunoassays
for the disease-causing PrP isoform (PrPSc) used limited
proteolysis to digest the precursor cellular PrP (PrPC).
Because the CDI is the only immunoassay that measures both
the protease-resistant and protease-sensitive forms of
PrPSc, we used the CDI to diagnose human prion disease. The
CDI gave a positive signal for PrPSc in all 10–24 brain
regions (100%) examined from 28 CJD patients. A subset of 18
brain regions from 8 patients with sporadic CJD (sCJD) was
examined by histology, immunohistochemistry (IHC), and the
CDI. Three of the 18 regions (17%) were consistently
positive by histology and 4 of 18 (22%) by IHC for the 8
sCJD patients. In contrast, the CDI was positive in all 18
regions (100%) for all 8 sCJD patients. In both gray and
white matter, 90% of the total PrPSc was protease-sensitive
and, thus, would have been degraded by procedures using
proteases to eliminate PrPC. Our findings argue that the CDI
should be used to establish or rule out the diagnosis of
prion disease when a small number of samples is available as
is the case with brain biopsy. Moreover, IHC should not be
used as the standard against which all other
immunodiagnostic techniques are compared because an
immunoassay, such as the CDI, is substantially more
sensitive.

snip...

Discussion

The clinical diagnosis of human prion disease is often
difficult until the patient shows profound signs of
neurologic dysfunction. It is widely accepted that the
clinical diagnosis must be provisional until a tissue
diagnosis either confirms or rules out the clinical
assessment. Before the availability of Abs to PrP, a tissue
diagnosis was generally made by histologic evaluation of
neuropil vacuolation. IHC with
anti-glial-fibrillary-acidic-protein Abs in combination with
H&E staining preceded the use of anti-PrP Ab staining.
Recently, the role of IHC in the diagnosis of scrapie in the
brains of eight clinically affected goats inoculated with
the SSBP1 prion isolate has been challenged (14). Thalamic
samples taken from seven of eight goats with scrapie were
positive for PrPSc by Western blotting but negative by IHC.
The eighth goat was negative by Western blotting and IHC.
Consistent with these findings in goats are the data
reported here, in which IHC of formalin-fixed,
paraffin-embedded human brain samples was substantially less
sensitive than the CDI.

The CDI was developed to quantify PrPSc in tissue samples
from mammals producing prions. Concerned that limited PK
digestion was hydrolyzing some or even most of the PrPSc, we
developed a CDI that does not require PK digestion. The CDI
revealed that as much as 90% of PrPSc is sPrPSc; thus, it
was being destroyed during limited proteolytic digestion
used to hydrolyze PrPC. sPrPSc comprises 80% of PrPSc in the
frontal lobe and in the white matter (Fig. 4).

The CDI detected HuPrPSc with a sensitivity comparable to
the bioassay for prion infectivity in Tg(MHu2M) mice (Fig.
1). The high sensitivity achieved by the CDI is due to
several factors (8, 10, 11, 15). First, both sPrPSc and
rPrPSc conformers are specifically precipitated by PTA
(Table 5) (8, 9). PTA has also been used to increase the
sensitivity of Western blots enabling the detection of
rPrPSc in human muscle and other peripheral tissues (16,
17). Second, a sandwich protocol was used with the
high-affinity MAR1 mAb (11) to capture HuPrPSc and
Eu-labeled 3F4 mAb to detect HuPrPSc (12). Third, the CDI
detects PrPSc by Ab-binding to native and denatured forms of
the protein and, therefore, does not depend on proteolytic
degradation of PrPC. We chose not to perform Western blots
on most of the samples used in this study because such
immunoblots require denaturation of the sample, which
eliminates measurement of the native signal corresponding to
PrPC (Table 5). Moreover, a comparison between the CDI and
Western blotting on brain samples from sCJD and variant CJD
patients showed that the CDI was 50- to 100-fold more
sensitive (15). Additionally, Western blots combined with
densitometry are linear over a 10- to 100-fold range of
concentrations, whereas the CDI is linear over a >104-fold
range. The CDI has been automated, which not only improves
accuracy and reproducibility (10) but also allows numerous
samples to be analyzed, as reported here. Western blots are
difficult to automate and are labor intensive.

Our studies show that only the CDI detected PrPSc in all
regions examined in 24 sCJD and 3 fCJD(E200K) brains (Figs.
2 and 6). Comparative analyses demonstrated that the CDI was
vastly superior to histology and IHC. When 18 regions of 8
sCJD and 2 fCJD(E200K) brains were compared, we discovered
that histology and IHC were unreliable diagnostic tools
except for samples from a few brain regions. In contrast,
the CDI was a superb diagnostic procedure because it
detected PrPSc in all 18 regions in 8 of 8 sCJD and 2 of 2
fCJD(E200K) cases (Tables 1 and 2).

Histologic changes in prion disease have been shown to
follow the accumulation of prions as measured by bioassay of
infectivity and by PrPSc accumulation (18–22). Because low
levels of PrPSc are not associated with neuropathologic
changes, some discrepancy between vacuolation and PrPSc was
expected. In contrast to histology, IHC measures PrP
immunostaining after autoclaving tissue sections exposed to
formic acid. Because IHC measures PrP, we expected the
sensitivity of this procedure might be similar to the CDI,
but that proved not to be the case. Whether exposure of
formic acid-treated tissue sections to elevated temperature
destroys not only PrPC but also sPrPSc and only denatures
rPrPSc remains to be determined. Such a scenario could
account for the lower sensitivity of IHC compared with CDI
or bioassay (Tables 1 and 2).

Studies of the white matter in CJD brains were particularly
informative with respect to the sensitivity of the CDI,
where PrPSc levels were low but readily detectable, 10- to
100-fold above the threshold value (Fig. 4). Because animal
studies have shown that PrPSc and infectivity are
transported anterogradely from one brain region to another
along neuroanatomical pathways (23–25), we expected to find
PrPSc in white matter as demonstrated by the CDI but not
IHC. Axonal transport of PrPSc is also suggested by
diffusion-weighted MRI scans of CJD cases, which show
high-intensity signals in analogous neocortical regions of
the right and left cerebral hemispheres (26). This symmetry
of neuroradiological abnormalities is consistent with spread
of PrPSc to the contralateral cortex by means of callosal
commissural pathways.

Most immunoassays that detect HuPrPSc do so only after
subjecting the sample to limited proteolysis to form PrP
27–30, followed by denaturation. Because the CDI measures
the immunoreactivity before and after denaturation to an
epitope that is exposed in native PrPC but buried in PrPSc,
limited proteolysis to eliminate PrPC is unnecessary. Assays
based on limited proteolysis underestimate the level of
PrPSc because they digest sPrPSc, which represents 80–90% of
PrPSc in CJD and scrapie brains (Fig. 4 and Table 5).

Gerstmann–Sträussler–Scheinker, an inherited human prion
disease, is caused by the P102L mutation in the PRNP gene.
In mice expressing the Gerstmann–Sträussler–Scheinker mutant
PrP transgene, the CDI detected high levels of sPrPSc(P101L)
as well as low levels of rPrPSc(P101L) long before
neurodegeneration and clinical symptoms occurred (9).
sPrPSc(P101L) as well as low concentrations of rPrPSc(P101L)
previously escaped detection (27). Whether a similar
situation applies in other genetic forms of prion disease,
sCJD, or variant CJD remains to be determined. Because most
of the PrPSc in the brains of sCJD patients is
protease-sensitive (Fig. 4), it is likely that the lower
sensitivity of IHC is due to its inability to detect sPrPSc.
Presently, we have no information about the kinetics of
either sPrPSc or rPrPSc accumulation in human brain. Limited
information on the kinetics of PrPSc accumulation in
livestock comes from studies of cattle, sheep, and goats
inoculated orally, but most of the bioassays were performed
in non-Tg mice (28–30) in which prion titers were
underestimated by as much as a factor of 104 (10).

The studies reported here are likely to change profoundly
the approach to the diagnosis of prion disease in both
humans and livestock (31–33). The superior performance of
the CDI in diagnosing prion disease compared to routine
neuropathologic examination and IHC demands that the CDI be
used in future diagnostic evaluations of prion disease.
Prion disease can no longer be ruled out by routine
histology or IHC. Moreover, the use of IHC to confirm cases
of bovine spongiform encephalopathy after detection of
bovine PrPSc by the CDI (10) seems an untenable approach in
the future. Clearly, the CDI for HuPrPSc is as sensitive or
more sensitive than bioassays in Tg(MHu2M) mice (Fig. 1).
Our results suggest that using the CDI to test large numbers
of samples for human prions might alter the epidemiology of
prion diseases. At present, there is limited data on the
frequency of subclinical variant CJD infections in the U.K.
population (34). Because appendixes and tonsils were
evaluated only by IHC, many cases might have escaped
detection (Tables 1 and 2). Equally important may be the use
of CDI-like tests to diagnose other neurodegenerative
disorders, such as Alzheimer's disease, Parkinson's disease,
and the frontotemporal dementias. Whether IHC underestimates
the incidence of one or more of these common degenerative
diseases is unknown. Moreover, CDI-like tests may help
determine the frequency with which these disorders and the
prion diseases occurs concomitantly in a single patient (35,
36).

Acknowledgements

snip...END

http://www.pnas.org/

Volume 349:1812-1820 November 6, 2003 Number 19

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

Background In patients with sporadic Creutzfeldt–Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt–Jakob disease is important for classification and diagnosis and perhaps even for prevention.

Methods We used a highly sensitive method of detection — involving the concentration of PrPSc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude — to search for PrPSc in extraneural organs of 36 patients with sporadic Creutzfeldt–Jakob disease who died between 1996 and 2002.

Results PrPSc was present in the brain tissue of all patients. In addition, we found PrPSc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrPSc in both spleen and muscle specimens. Patients with extraneural PrPSc had a significantly longer duration of disease and were more likely to have uncommon molecular variants of sporadic Creutzfeldt–Jakob disease than were patients without extraneural PrPSc.

Conclusions Using sensitive techniques, we identified extraneural deposition of PrPSc in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt–Jakob disease. Extraneural PrPSc appears to correlate with a long duration of disease.

Source Information

From the Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital of Zurich, Zurich, Switzerland.

Dr. Glatzel and Mr. Abela contributed equally to the article.
Address reprint requests to Dr. Aguzzi at the Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12, CH-8091 Zurich, Switzerland, or at adriano@pathol.unizh.ch .

http://content.nejm.org/cgi/

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A. Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano Aguzzi, MD, PhD 6, Herbert Budka, MD 1 *
1Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria

2National Institute of Psychiatry and Neurology, Budapest, Hungary

3Department of Pathology, School of Medicine, Federal University of Rio de Janeiro

4Department of Neurology, School of Medicine, Federal University of Rio de Janeiro

5Department of Neurology, School of Medicine, Federal University of Sao Paulo, Brazil

6Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland

email: Herbert Budka (h.budka@akh-wien.ac.at )

*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J, Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:

European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523
EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837

Abstract

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrPSc in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become prominent when PrPC is abundantly available as substrate, as in inclusion body myositis muscle.

--------------

Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11 September 2003
Digital Object Identifier (DOI)

10.1002/ana.10813 About DOI

http://www3.interscience.wiley.com/

AS Professor Aguzzi kindly put it most recently ;

107

Vet Pathol 42:107 108 (2005)

Letters to the Editor

Editor:

Absence of evidence is not always evidence of absence. In the article Failure to detect prion protein (PrPres) by immunohistochemistry in striated muscle tissues of animals experimentally inoculated with agents of transmissible spongiform encephalopathy, recently published in Veterinary Pathology (41:78 81, 2004), PrPres was not detected in striated muscle of experimentally infected elk, cattle, sheep, and raccoons by immunohistochemistry (IHC). Negative IHC, however, does not exclude the presence of PrPSc. For example, PrPres was detected in skeletal muscle in 8 of 32 humans with the prion disease, sporadic Creutzfeldt-Jakob disease (CJD), using sodium phosphotungstic acid (NaPTA) precipitation and western blot.1 The NaPTA precipitation, described by Wadsworth et al.,3 concentrates the abnormal isoform of the prion, PrPres, from a large tissue homogenate volume before western blotting. This technique has increased the sensitivity of the western blot up to three orders of magnitude and could be included in assays to detect PrPres. Extremely conspicuous deposits of PrPres in muscle were detected by IHC in a recent case report of an individual with inclusion body myositis and CJD.2 Here, PrPres was detected in the muscle by immunoblotting, IHC, and paraf- fin-embedded tissue blot. We would therefore caution that, in addition to IHC, highly sensitive biochemical assays and bioassays of muscle are needed to assess the presence or absence of prions from muscle in experimental and natural TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland
References

1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812 1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121 125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171 180,
2001...///

EMBO reports AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie

http://www.emboreports.org/

Watson Meldrum et al Scrapie agent in muscle - Pattison I A (1990)

Veterinary record, 20 January 1990. p.68

http://www.bseinquiry.gov.uk/files/yb/1990/01/19009001.pdf

===============================

GREETINGS AGAIN APHIS ET AL,

FURTHERMORE, WE HAVE FAILED TO EVEN STOP THE SRMs FROM WHOLE CUTS OF BONELESS BEEF IMPORTED FROM CANADA IN THE VERY ONSET OF THE NEW BSE MRR (MINIMAL RISK REGION). THIS IS THE VERY REASON I HAVE SAID TIME AND TIME AGAIN THAT BY THIS ADMINISTRATION ABANDONING THE BSE GBR RISK ASSESSMENTS BECAUSE THEY DID NOT LIKE THE ASSESSMENT OF BSE GBR III, AND ADHERING TO A NEW BSE MRR POLICY THAT WAS DESIGNED NOT FOR HUMAN HEALTH, BUT ONLY FOR COMMODITIES AND FUTURES, WILL FURTHER EXPOSE NEEDLESSLY MILLIONS AND MILLIONS OF HUMANS AND ANIMALS VIA THE FREE TRADING OF ALL STRAINS OF TSE GLOBALLY. references as follow ;

Wisconsin Firm Recalls Beef Products

Recall Release CLASS II RECALL

FSIS-RC-032-2005 HEALTH RISK: LOW

Congressional and Public Affairs

(202) 720-9113

Steven Cohen

WASHINGTON, Aug. 19, 2005 - Green Bay Dressed Beef, a Green Bay, Wis., establishment, is voluntarily recalling approximately 1,856 pounds of beef products that may contain portions of the backbone from a cow just over 30 months old, the U.S. Department of Agriculture's Food Safety and Inspection Service announced today. The product was from a cow imported directly for slaughter from Canada.

Based on information provided by Canada, the products subject to this Class II recall are from a cow that is approximately one month older than the 30-month age limit. Both ante-mortem and post-mortem inspection were done on the cow in question. FSIS inspection program personnel determined the cow to be healthy and fit for human food. FSIS' designation of this recall as Class II is because it is a situation where there is a remote probability of adverse health consequences from the use of the product.

FSIS learned about this as a result of a Canadian audit of their health certificate that accompanied the imported cow. Prior to slaughter, the health certificate accompanying the cow was presented to the establishment, and it appeared complete and accurate. However, a subsequent audit of information related to the health certificate by Canadian officials found that it was not accurate. Action has been taken by Canadian Food Inspection Agency officials in response to findings from the audit.
The products subject to recall are:

Five boxes of 243 lb. vacuum pouched packages of "American Foods Group, NECKBONE UNTRIM'D, USDA CHOICE OR HIGHER" with the case code of 77333;
One box of 50 lb. vacuum pouched package of "American Foods Group, SHORTLOIN 2X2, USDA SELECT OR HIGHER" with the case code of 75231;
One box of 60 lb. vacuum pouched package of "American Foods Group, SHORTLOIN 2X2, USDA CHOICE OR HIGHER" with the case code of 75060;
Five boxes of 258 lb. vacuum pouched packages of "Dakota Supreme Beef, SHORTLOIN 0X11/4, USDA SELECT OR HIGHER" with the case code of 75442;
Sixteen boxes of 811 lb. vacuum pouched packages of "American Foods Group, BLADE BI N/O CHUCK, USDA CHOICE OR HIGHER" with the case code of 75955;
Nine boxes of 435 lb. vacuum pouched packages of "American Foods Group, BLADE BI N/O CHUCK, USDA SELECT OR HIGHER" with the case code of 75952.
Each box bears the establishment number "410" inside the USDA seal of inspection. The products were produced on August 4, and were distributed to wholesale distributors in Pennsylvania, Florida, Illinois, Maryland, Minnesota and Wisconsin.

Under the interim final rules FSIS implemented on January 12, 2004, certain specified risk materials must be removed from all cattle depending on the age of the animal. On this animal all specified risk materials for cattle 30 months and over were removed, with the exception of the vertebral column. At the time of slaughter, the animal was certified to be under 30 months of age and removal of the vertebral column was not required. A subsequent audit determined the animal was just over 30 months of age; therefore, the vertebral column is required to be removed. This is the reason for the recall of the selected products.

Consumers with questions about the recall may contact Sally VandeHei, Executive Assistant at 1-877-894-3927. National media with questions may contact Jim Mulhern at (202) 496-2468. Local media with questions may contact Susan Finco at (920) 965-7750 ext.158.

Consumers with other food safety questions can phone the toll-free USDA Meat and Poultry Hotline at 1-888-MPHotline (1-888-674-6854). The hotline is available in English and Spanish and can be reached from 10 a.m. to 4 p.m. (Eastern Time), Monday through Friday. Recorded food safety messages are available 24 hours a day.

Sample Product Labels: These are similar to, but not identical to, labels on the recalled product.

#

USDA Recall Classifications

Class I This is a health hazard situation where there is a reasonable probability that the use of the product will cause serious, adverse health consequences or death.

Class II This is a health hazard situation where there is a remote probability of adverse health consequences from the use of the product.

Class III This is a situation where the use of the product will not cause adverse health consequences.

http://www.fsis.usda.gov/News_&_Events/Recall_032_2005_Release/index.asp

:
Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA
[TSS SUBMISSION 11/03/2003 01:19 PM To: regulations@aphis.usda.gov ]

https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenDocument&AutoFramed

THE BSE MRR POLICY SHOULD BE ABOLISHED/REPEALED IMMEDIATELY AND THE BSE GBR RISK ASSESSMENTS AND POLICY SHOULD BE STRICTLY ENFORCED AND FURTHER ENHANCED TO INCLUDE CWD AND ALL TSEs...TSS

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA)

Publication date: 20 August 2004

Adopted July 2004 (Question N° EFSA-Q-2003-083)

Report

Summary

Summary of the Scientific Report

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.

A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.eu.int/science/efsa_scientific_reports/gbr_assessments/573_en.html

From: Terry S. Singeltary Sr. [flounder@wt.net]

Sent: Tuesday, July 29, 2003 1:03 PM

To: fdadockets@oc.fda.gov

Cc: ggraber@cvm.fda.gov; Linda.Grassie@fda.gov; BSE-L

Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]

Greetings FDA,

snip...

PLUS, if the USA continues to flagrantly ignore the _documented_ science to date about the known TSEs in the USA (let alone the undocumented TSEs in cattle), it is my opinion, every other Country that is dealing with BSE/TSE should boycott the USA and demand that the SSC reclassify the USA BSE GBR II risk assessment to BSE/TSE GBR III 'IMMEDIATELY'. for the SSC to _flounder_ any longer on this issue, should also be regarded with great suspicion as well. NOT to leave out the OIE and it's terribly flawed system of disease surveillance. the OIE should make a move on CWD in the USA, and make a risk assessment on this as a threat to human health. the OIE should also change the mathematical formula for testing of disease. this (in my opinion and others) is terribly flawed as well. to think that a sample survey of 400 or so cattle in a population of 100 million, to think this will find anything, especially after seeing how many TSE tests it took Italy and other Countries to find 1 case of BSE (1 million rapid TSE test in less than 2 years, to find 102 BSE cases), should be proof enough to make drastic changes of this system. the OIE criteria for BSE Country classification and it's interpretation is very problematic. a text that is suppose to give guidelines, but is not understandable, cannot be considered satisfactory. the OIE told me 2 years ago that they were concerned with CWD, but said any changes might take years. well, two years have come and gone, and no change in relations with CWD as a human health risk. if we wait for politics and science to finally make this connection, we very well may die before any decisions or changes are made. this is not acceptable. we must take the politics and the industry out of any final decisions of the Scientific community. this has been the problem from day one with this environmental man made death sentence. some of you may think i am exaggerating, but you only have to see it once, you only have to watch a loved one die from this one time, and you will never forget, OR forgive...yes, i am still very angry... but the transmission studies DO NOT lie, only the politicians and the industry do... and they are still lying to this day...TSS

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

GREETINGS AGAIN APHIS ET AL,

Moving on to the theory that BSE agent is not in blood. THIS is what they use to think with nvCJD. However the nvCJD agent has now been detected and transmitted the TSE agent by blood. nvCJD is the BSE agent that has transmitted to humans. nvCJD is human BSE. so if nvCJD transmits by blood, why not BSE? with the limited testing to date, the limited sensitivity of the detection of the BSE/TSE agent blood to date, i would not be so sure that the BSE/TSE agent does not transmit by blood. just recent Ag. Comm. Johanns stated that they would not address the blood issue being fed to cattle. a foolish and careless mistake. but typical.

we now have detected new atypical strains of the BSE/TSE agent in cattle in many countries i.e. Japan, France, Belgium, Germany, and Italy. In the Italian study of BASE, a new? TSE in cattle they have discovered that is not like the nvCJD, but very similar to sporadic CJD. They have detected 2 such cattle at printing of this study March 2, 2004. Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease PNAS.

MY point is that with these new atypical TSEs showing up in cattle, sheep and goats, we don't know if blood and other tissues transmits the disease. THE SRM list may and should be reevaluated. WE know BSE is in the USA, but we also know that in the studies of Mission Texas, where USA suffolk scrapie sheep were inoculated into USA cattle, the PRION agent that was produced did not look like the UK BSE strain. so why would all CJD cases in the USA look like the UK human BSE i.e. nv/v CJD?

WHY wait and expose millions and millions needlessly as in the past with previous TSE blunders, why wait to act. why not act first with what we know, which is very concerning, then as science evolves, go from there. references as follow ;

UK Strategy for Research and
Development on Human and Animal
Health Aspects of Transmissible
Spongiform Encephalopathies
2005-2008

snip...

3.2 Tackling the spread of infection

3.2.1 The species barrier and the carrier state

3.2.1.1 The possibility of ‘carrier’ states in animals and humans, and our present inability to identify them, pose a potential threat to public and animal health. The susceptibility of humans to BSE infection, and the ability of the disease to remain clinically silent for many years, is of major concern to DH. Although the death of a UK blood donor from vCJD in 1999 three years after making the donation and the subsequent death from vCJD of the recipient in 2003 have not been causally linked, transmission of infection through blood transfusion is the most likely explanation327. The case heightens concerns that ‘carriers’ could be transmitting the disease through blood, tissue and organ donation or by contaminating surgical instruments when undergoing surgery. DH will continue to support research to detect infectious prions in human tissue, to investigate the decontamination of surgical instruments and to develop measures to protect blood supplies.

3.2.1.2 Animal models of some TSEs have detected infectivity in blood. Experiments, which have involved transfusing large volumes of blood from infected sheep to healthy recipient sheep, have demonstrated that infectivity can be transmitted by blood transfusion. A central part of DH policy in this area has been the leucodepletion of blood donations and the efficacy of this technology can now be tested in sheep.

snip...

4.5.4 In 1987, epidemiological studies of BSE cases identified meat and bone meal as the probable means by which the disease was being spread. In an attempt to prevent further infections a ban on incorporating ruminant protein in ruminant feed was introduced in July 1988. Due to the long incubation period associated with this disease the efficacy of this control measure was not immediately apparent. As time passed it became clear from the number of cases born after the ban that it was not wholly effective.

4.5.5 Epidemiological analysis of these cases showed that a high proportion of them occurred in areas where the pig population was high. This observation, coupled with research data that showed that only a very small dose of the infective material was needed to cause disease in cattle, led to the conclusion that cross-contamination of feed was occurring.

4.5.6 Since 1988, increasingly stringent feed controls have been put in place. Key amongst these have been:

• the ban on the use of specified bovine offal in all animal feed (September 1990);

• the ban on feeding any farmed animal, including horses and fish, with mammalian meat and bone meal. (This began in March 1996 but following this ban there was a recall scheme and the date from which the ban was considered to be fully effective is regarded as being 1 August 1996);

62

v6.1

• EU-wide controls on feed which extended the ban to include all processed animal protein, including that derived from birds and fish (implemented in the UK from 1 August 2001).

4.5.7 As illustrated in fig. [ ] these later measures have reduced the spread of BSE. However, they have not been one hundred per cent effective. As at 31st December 2003 there had been 81 cases of BSE in animals born since 1 August 1996 in the UK.

full text 91 pages;

http://www.mrc.ac.uk/pdf-about-tse_uk_strategy_june2005.pdf

GREETINGS AGAIN APHIS ET AL,

JUST what about those old studies at Mission Texas and the atypical TSE in cattle? would it not be prudent for human health purpose, the question that, with all the atypical TSEs showing up in animal and man in different countries, the fact that most all of these TSEs transmit as freely or not as freely as BSE (depending whom you have witnessed die from this agent either directly or indirectly via a multitude of potential routes and sources) to primates. would it not be prudent to ask yourself if some if not all of these sporadic CJDs might be a by-product of these TSEs either directly or indirectly via a multitude of proven routes and sources in animal studies? it is unethical for human transmission studies considering the fact that the agent is 100% fatal, slow, but fatal. there has been no sound science in any of the recent decisions in the USA in regards to BSE/TSE human or animal, all one has to do is look at TEXAS, the mad cow that got away, the stumbling and staggering one that NO TSE TEST AT ALL was done, ordered rendered, and then the infamous positive, positve, secret positive, inconclusive (NO WB), negative, 8 month delay, then the 'Fong Effect' took place, THEN FINALLY CONFIRMED SOME 8 MONTHS LATER IN WEYBRIDGE.

Finally recently, another inconclusive that took place that sat untested on some shelf for about 4 months while the Texas mad cow blunder was going on. the tissues of this cow this time were preserved in preservative as to render any WB for further confirmation, what i called the 'FONG SYNDROME' or the 'end around' the WB ordered previously by the Honorable Phyllis Fong of the OIG. Politics at its finest, to hell with human health. WE find now that 9,200 USA POTENTIAL MAD COWS IN JUNE 2004 ENHANCED COVER-UP SURVEILLANCE PROGRAM WENT UNTESTED WITH NO RAPID TEST OR WB, ONLY IHC, the test that fails the most, that is very unreliable as noted above in my submission by Prusiner et al and other scientist.

NOW, back to Mission, Texas ;

> > Differences in tissue distribution could require new regulations

> > regarding specific risk material (SRM) removal.

snip...end

full text 33 PAGES ;

http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

It was, however, performed in the USA in 1979, when it was shown that cattle
inoculated with the scrapie agent endemic in the flock of Suffolk sheep at
the United States Department of Agriculture in Mission, Texas, developed a
TSE quite unlike BSE. 32 The findings of the initial transmission, though
not of the clinical or neurohistological examination, were communicated in
October 1988 to Dr Watson, Director of the CVL, following a visit by Dr
Wrathall, one of the project leaders in the Pathology Department of the CVL,
to the United States Department of Agriculture. 33 The results were not
published at this point, since the attempted transmission to mice from the
experimental cow brain had been inconclusive. The results of the clinical
and histological differences between scrapie-affected sheep and cattle were
published in 1995. Similar studies in which cattle were inoculated
intracerebrally with scrapie inocula derived from a number of
scrapie-affected sheep of different breeds and from different States, were
carried out at the US National Animal Disease Centre. 34 The results,
published in 1994, showed that this source of scrapie agent, though
pathogenic for cattle, did not produce the same clinical signs of brain
lesions characteristic of BSE.

http://www.bseinquiry.gov.uk/

1: J Infect Dis. 1994 Apr;169(4):814-20.

Intracerebral transmission of scrapie to cattle.

Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM,
Robinson MM.

USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA
50010.

To determine if sheep scrapie agent(s) in the United States would induce a
disease in cattle resembling bovine spongiform encephalopathy, 18 newborn
calves were inoculated intracerebrally with a pooled suspension of brain
from 9 sheep with scrapie. Half of the calves were euthanatized 1 year after
inoculation. All calves kept longer than 1 year became severely lethargic
and demonstrated clinical signs of motor neuron dysfunction that were
manifest as progressive stiffness, posterior paresis, general weakness, and
permanent recumbency. The incubation period was 14-18 months, and the
clinical course was 1-5 months. The brain from each calf was examined for
lesions and for protease-resistant prion protein. Lesions were subtle, but a
disease-specific isoform of the prion protein was present in the brain of
all calves. Neither signs nor lesions were characteristic of those for
bovine spongiform encephalopathy.

MeSH Terms:
Animals
Brain/microbiology*
Brain/pathology
Cattle
Cattle Diseases/etiology*
Cattle Diseases/pathology
Encephalopathy, Bovine Spongiform/etiology*
Encephalopathy, Bovine Spongiform/pathology
Immunoblotting/veterinary
Immunohistochemistry
Male
Motor Neurons/physiology
Prions/analysis
Scrapie/pathology
Scrapie/transmission*
Sheep
Sleep Stages
Time Factors
Substances:
Prions

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8133096&dopt=Citation

Intracerebral transmission of scrapie to cattle FULL TEXT PDF;

SNIP...

Discussion

WE conclude that American sources of sheep scrapie are transmissible to
cattle by direct intracerebral inoculation but the disease induced is NOT
identical to BSE as seen in the United Kingdom. While there were
similarities in clinical signs between this experimental disease and BSE,
there was no evidence of aggressiveness, hyperexcitability, hyperesthesia
(tactile or auditory), or hyperemetria of limbs as has been reported for BSE
(9). Neither were there extensive neurologic lesions, which are primary for
BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis
and gliosis. Although some vacuolation of neuropil, chromotolysis in
neurons, and gliosis were seen in the brains of some affected calves, these
were industinguishable from those of controls. Vacuolated neurons in the red
nucleus of both challenged and normal calves were considered normal for the
bovines as previously described (50).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, and
the amount of PrP-res positively related to the length of the incubation...

snip...

WE also conclude from these studies that scrapie in cattle MIGHT NOT BE RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGEST
THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is
necessary to make a definitive diagnosis. THUS, undiagnosed scrapie
infection could contribute to the ''DOWNER-COW'' syndrome and could be
responsible for some outbreaks of transmissible mink encephalopathy proposed
by Burger and Hartsough (8) and Marsh and harsough (52). ...

snip...

Multiple sources of sheep affected with scrapie and two breeds of cattle
from several sources were used inthe current study in an effort to avoid a
single strain of either agent or host. Preliminary results from mouse
inoculations indicate multiple strains of the agent were present in the
pooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by using
intracerebral, intramuscular, subcutaneous, and oral routes of inoculation
of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1
affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48
months after inoculation. Signs were disorientation, incoordination, a
stiff-legged stilted gait, progressive difficulty in rising, and finally in
terminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattle
similarly inoculated with brain tissue from a goat with scrapie exhibited
similar signs 27 and 36 months after incoluation. Clinical courses were 43
an 44 days. Brain lesions of mild gliosis and vacuolation and mouse
inoculation data were insufficient to confirm a diagnosis of scrapie. This
work remained controversial until recent examination of the brains detected
PrP-res in all 3 cattle with neurologic disease but in none of the
unaffected cattle (62). Results of these studies are similar to ours and
underscore the necessity of methods other than histopathology to diagnose
scrapie infection in cattle. We believe that immunologic techniques for
detecting PrP-res currently provide the most sensitive and reliable way to
make a definitive diagnosis...

http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf

Visit to USA ... info on BSE and Scrapie

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

GREETINGS AGAIN APHIS ET AL,

COMMENTING ON THE SRMs AND THE REMOVAL OF SRMs. I FIND IT DISTURBING THAT RECENTLY, AFTER A LONG BATTLE FOR DOCUMENTS VIA THE FOIA THAT THE MEDIA AND CONSUMERS UNION REQUESTED, THAT DURING THE JUNE 2004 ENHANCED BSE SURVIELLANCE PROGRAM, THAT OVER 1,000 CITATIONS WERE ISSUED FOR VARIOUS SRM VIOLATIONS, BUT YET IT TOOK THE FOIA TO GET THIS TO THE PUBLIC.

WOULD it not be likely that from some of these noncompliance reports that
indeed some breaches led to some potential tainted materials to enter the
animal/human feed chain?

ALL we have heard about in the last 7 years or better, well, since the
12/14/97 partial and voluntary ruminant-to-ruminant feed ban is that the
feed ban is working, tripple fire walls, no ruminant protein entering the
animal feed chain. along with this was a constant barage of 'no mad cow
disease in the USA'. then we find this 12 year old TEXAS cow that was
infected from tainted feed some time in that 12 year period. so, the NE
TEXAS CJD cluster, where it was stated that NO mad cow was in the USA or
TEXAS at that time frame, was in fact not true.

SINCE some 460 of these occurred because slaughter plants did not have an
adequate plan for dealing with BSE in their plant's food safety plan, as
required by the USDA, the analysis showed, and of those 460 violations, 60
percent described plans that contained no mention of BSE at all. then again,
would it not be very possible that indeed some potentially tainted material
of a BSE or atypical TSE DID enter the animal feed chain, thus later some of
those animals entering the human food chain.

WHAT about the SRM violations? Violations of rules about the removal and
handling of specified risk material (SRMs) occurred at 131 plants in at
least 35 states. SRMs are the high-risk materials, such as brains and spinal
cords, most likely to be infectious. More than 30 percent of the NRs
analyzed were due to either improperly handling or removing SRMs. Could this
not have also led to potentially BSE/TSE tainted materials entering the
animal/human food chain?

In 10 percent of the NRs analyzed, plants incorrectly identified the age of
cattle. THIS also could have led to tainted BSE/TSE SRM materials entering
the animal/human food chain.

IN my opinion, this could have led to many feed discrepancies and should
HAVE been reported to the public, without the media having to request this
data via FOIA. I think in the future it would be best if the NRs
(non-compliance reports) were made easily available to the public in there
feed enforement reports. ...

FOR IMMEDIATE RELEASE
AUGUST 18, 2005
5:25 PM CONTACT: Public Citizen
(202) 588-1000

Evidence of Weak Meat Inspection Program Found in Nearly a Thousand
Violations of Mad Cow Rules at Slaughter Plants
Noncompliance Records Show Plants Failed to Follow Regulations

WASHINGTON - August 18 - In stark contrast to the public relations message
touted by the U.S. Department of Agriculture (USDA) and the beef industry
that the U.S. regulatory system is adequate to prevent the spread of mad cow
disease, an analysis released today by the consumer group Public Citizen
found significant lapses in the industry's compliance with federal rules.
The analysis stems from a December 2004 Freedom of Information Act (FOIA)
request from Public Citizen to the USDA for all "noncompliance records"
(NRs) related to bovine spongiform encephalopathy (BSE). Public Citizen
received copies of 829 records on Aug. 15.

More than half the violations (460) occurred because slaughter plants did
not have an adequate plan for dealing with BSE in their plant's food safety
plan, as required by the USDA, the analysis shows. Of those 460 violations,
60 percent described plans that contained no mention of BSE at all.
"The fact that 60 percent of the violations were due to a failure to even
mention BSE or risk materials such as brains and spinal cords is
significant," said Patty Lovera, deputy director of Public Citizen's food
program. "If officials running a meat plant cannot be bothered to recognize
the risk of BSE when writing their safety plan, how much of a priority is it
in daily operations and training of staff?"

The analysis also found that:

Violations of rules about the removal and handling of specified risk
material (SRMs) occurred at 131 plants in at least 35 states. SRMs are the
high-risk materials, such as brains and spinal cords, most likely to be
infectious. More than 30 percent of the NRs analyzed were due to either
improperly handling or removing SRMs. The SRM ban is considered a critical
firewall in protecting the food supply from BSE.

The violations described in the NRs occurred from January 2004 through March
2005. This shows that the problems in the plants persisted long after plants
should have adapted to new rules issued in January 2004 after the discovery
of the first case of BSE in the United States.

In 10 percent of the NRs analyzed, plants incorrectly identified the age of
cattle. Properly determining the age of cattle is a crucial step in proper
SRM removal because the definition of SRMs is dependent on age; in cattle
older than 30 months, there is a greater likelihood that SRM will carry BSE
and therefore must be removed. Accurately identifying the head, spine and
carcass of cattle by age is necessary to ensure that all SRMs are removed as
the carcass moves down the slaughter line.

"These enforcement records only increase our concerns about how easily
potentially infected cattle are bypassing inspection points at
slaughterhouses, creating one more opportunity for infected meat to slip
through the system," said Tony Corbo, legislative representative of Public
Citizen's food program. "We're approaching the two-year mark of our first
case of mad cow in the United States, yet the government is still lagging
behind on protecting consumers."

Public Citizen sent the FOIA request to the USDA in December 2004 after the
chairman of the USDA meat inspectors union, Stan Painter, raised concerns
about the agency's policy for ensuring that cattle age is properly
determined. Instead of investigating whether the policy was adequate, the
agency opened a misconduct investigation on Painter. The investigation was
closed this week, shortly after Public Citizen received the documentation,
which contained more than 80 records of plants improperly identifying cattle
age.

###

http://www.commondreams.org/news2005/0819-02.htm

Daily Update

On August 19, 2005, no inconclusive test results were reported.

National Veterinary Services Laboratory (NVSL) Immunohistochemistry (IHC) Testing Summary
The BSE enhanced surveillance program involves the use of a rapid screening test, followed by confirmatory testing for any samples that come back "inconclusive." The weekly summary below captures all rapid tests conducted as part of the enhanced surveillance effort. It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing. This was done to ensure that samples inappropriate for the rapid screen test were still tested, and also to monitor and improve upon IHC testing protocols. Of those 9,200 routine tests, one test returned a non-definitive result on July 27, 2005. That sample underwent additional testing at NVSL, as well as at the Veterinary Laboratories Agency in Weybridge, England, and results were negative.

To view the IHC testing numbers from 1990 through 2004, click on the following link:

http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure2f.html

GREETINGS AGAIN,

THE APHIS/USDA/FSIS et al has failed the public terribly with there industry friendly approach to erradicate BSE/TSE aka mad cow disease in the USA. the 8/4/97 partial and voluntary ruminant to rumiant feed ban program and there cealed borders were, have been and still are a joke. by sleeping with the industry, this administration has needlessly exposed millions and millions to the TSE agent at home, and abroad. THE fact that the first documented home grown mad cow came from TEXAS, the TAHC/USDA et al had already covered up one suspect mad cow and rendered with no test at all, but for the Honorable Phyllis Fong of the OIG to have the courage to go around the Agriculture Secretary Johann to have it confirmed in Weybridge, England (it would have never been confirmed any other way), but to have her do this, was truely a coup of sorts, one of the most couragous I have seen in a while by any person of our Government. BUT to finally have this mad cow confirmed, and then have the Secretary of Agriculture Johann not give praise to her for finally confirming this deadly disease in a home grown case, but to have him want her head for it raises serious serious doubts of the over June 2004 Enhanced BSE program where they claim some 500,000 cows have now been tested. NOW, we find indeed this was the case. WE find now that of those 500,000+ cows, 9,200 of them did not have rapid testing at all, no WB, only IHC. The least likely to find a case of BSE/TSE, the one that fails the most. a proven hit and miss test. Dr. Detwiler tried to tell them this in 2003 ;

USDA 2003

We have to be careful that we don't get so set in the way we do things that
we forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.

snip.............

Dr. Detwiler: It seems a good idea, but I'm not aware of it.
Another important thing to get across to the public is that the negatives
do not guarantee absence of infectivity. The animal could be early in the
disease and the incubation period. Even sample collection is so important.
If you're not collecting the right area of the brain in sheep, or if
collecting lymphoreticular tissue, and you don't get a good biopsy, you
could miss the area with the PRP in it and come up with a negative test.
There's a new, unusual form of Scrapie that's been detected in Norway. We
have to be careful that we don't get so set in the way we do things that we
forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.

snip...

FULL TEXT;

Completely Edited Version
PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

Greetings again APHIS et al,

THE June 2004 Enhanced BSE/TSE surveillance program was a terrible failure, other than to prove just how bad the situation is in the USA, and how out of control the Federal Government is in trying to cover it up. THE OIG should hold an inquiry into this program. THE BSE MRR policy should be dismantled, and the USA BSE GBR risk assessment should be immediately raised to BSE GBR IV.
THE International guidelines for trade in animal and animal products which are developed by the World Organization for Animal Health (formerly known as the Office International des Epizooties (OIE)), is and has been terrible flawed. ALL one has to do is to look at the countries that have gone by there very minimal guidelines, most all went on to develop BSE. IT would be nice if the OIE et al would define “controlled BSE-risk country” or “effectively enforced ban”. The USA and North America have neither. THIS has been proven time and time again via the GAO, OIG and the European EFSA BSE-risk assessments of North American countries. Many Countries have not even reported there first case of BSE yet, and many countries have not even produced a risk analysis for BSE. A fine example is Mexico and Canada. I pointed out about Canada above, but now lets look at Mexico, which is also a BSE GBR III country. IN Mexico, they are NOT even required to remove SRM;

Working Group Report on

the Assessment of the Geographical BSE-Risk (GBR) of MEXICO

2004

Specified Risk Material (SRM) and fallen stock There is no SRM-ban. SRM is normally destined for human consumption. According to the CD, fallen stock from pasture and diseased animals are incinerated and not rendered.

Conclusion on the ability to avoid recycling In light of the above information, it has to be assumed that the BSE agent, should it have entered Mexico, could have been recycled and potentially amplified.

snip...

In view of the above - described consideration the combination of the very / extremely
high external challenges with a very unstable system makes the occurrence of an
internal challenge likely in Mexico from approximately 1993 onwards.

4.2 Risk that BSE infectivity entered processing

It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’
MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late
1990’s). The high level of external challenge is maintained throughout the reference
period, and the system has not been made stable, leading to increased internal

4.3 Risk that BSE infectivity was recycled and propagated

It is likely that BSE infectivity was recycled and propagated from approximately
1993. The risk has since grown consistently due to a maintained internal and external
challenge and lack of a stable system.

5. CONCLUSION ON THE GEOGRAPHICAL BSE - RISK

5.1 The current GBR as function of the past stability and challenge
The current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed
that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent.

snip...end

http://www.efsa.eu.int/science/efsa_scientific_reports/gbr_assessments/scr_annexes/566/sr04_biohaz02_mexico_report_annex_en1.pdf

MORE ON THOSE USA BSE/TSE SEALED BORDERS

Aug. 22, 2005, 12:35AM

Mexican cattle business linked to drug cartels

Animals sold to Texas ranchers by 2 companies can be seized

By MICHAEL HEDGES

Copyright 2005 Houston Chronicle Washington Bureau

WASHINGTON - The Treasury Department, trying to block an elaborate money-laundering scheme, has announced that two Mexican cattle companies are fronts for drug-trafficking cartels.
The action means that cattle sold by the companies to Texas ranchers after Friday's announcement are subject to seizure by the federal government, said a high-ranking Treasury official who asked not to be named.

"Cattle already purchased and owned before the companies were identified as tied to the drug cartels are not going to suddenly be blocked," the official said.

The Treasury Department plans to inform cattle associations and other groups later this week of the action taken against the Mexican companies, officials said. The Treasury also will provide other information, such as the brands used by the cattle companies linked to the drug cartels.
For now, buyers are expected to practice due diligence when purchasing cattle.

Two Mexican drug cartels were named in the Treasury Department's statement, the Arriola Marquez organization and the Arellano Felix cartel based in Tijuana. The Arriola Marquez group, based in Mexico's Chihuahua state, is linked to Mexican drug kingpin Joaquin "El Chapo" Guzman, the department said. Guzman leads one of the factions fighting for control of Nuevo Laredo and its smuggling routes into Texas, officials have said.

snip...

It is not clear how many Mexican cattle owned by companies linked to drug cartels had been sold in Texas, said government officials and Texas cattle raisers.

snip...end

http://www.chron.com/cs/CDA/ssistory.mpl/topstory/3319609

I do not think that USDA et al used rapid test, WB or IHC on any of these cattle for BSE/TSE.
TRIPLE firewalls and SEALED borders, I dont think so.

FINALLY COMMENTING ON THE UK BSE/nv/vCJD ONLY THEORY. there are different strains of TSE showing up in cattle, sheep, and goats. nvCJD has been documented in a 74 year old and the young are dying of sporadic CJD. one of the new atypical strains of TSE in cattle 'BASE' does not look like nvCJD in humans, but it looks very similar to sporadic CJD. also, Asante/Collinge et al have shown that BSE can propagate as nvCJD AND sporadic CJD. to continue to flounder and ignore all of this as in the past, to continue to cater to big beef, big feed will only allow this agent to further amplify and spread. there is more to this agent than the mad cow hamburger. AS long as the BSE MRR policy is in effect, the agent will continue to spread globally. references as follow ;

Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice

Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Jennifer Buckell, Sebastian Brandner, Jonathan D. F. Wadsworth and John Collinge

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, London WC1N 3BG, UK

Correspondence
John Collinge
j.collinge@prion.ucl.ac.uk

Distinct prion strains can be distinguished by differences in incubation period, neuropathology and biochemical properties of disease-associated prion protein (PrPSc) in inoculated mice. Reliable comparisons of mouse prion strain properties can only be achieved after passage in genetically identical mice, as host prion protein sequence and genetic background are known to modulate prion disease phenotypes. While multiple prion strains have been identified in sheep scrapie and Creutzfeldt-Jakob disease, bovine spongiform encephalopathy (BSE) is thought to be caused by a single prion strain. Primary passage of BSE prions to different lines of inbred mice resulted in the propagation of two distinct PrPSc types, suggesting that two prion strains may have been isolated. To investigate this further, these isolates were subpassaged in a single line of inbred mice (SJL) and it was confirmed that two distinct prion strains had been identified. MRC1 was characterized by a short incubation time (110±3 days), a mono-glycosylated-dominant PrPSc type and a generalized diffuse pattern of PrP-immunoreactive deposits, while MRC2 displayed a much longer incubation time (155±1 days), a di-glycosylated-dominant PrPSc type and a distinct pattern of PrP-immunoreactive deposits and neuronal loss. These data indicate a crucial involvement of the host genome in modulating prion strain selection and propagation in mice. It is possible that multiple disease phenotypes may also be possible in BSE prion infection in humans and other animals.

=======================

Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco ||, and Maria Caramelli *
*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.

--------------------------------------------------------------------------------

C.C. and G.Z. contributed equally to this work.
||To whom correspondence should be addressed.
E-mail: salvatore.monaco@mail.univr.it.
www.pnas.org/cgi/doi/10.1073/pnas.0305777101

=====================================

eurobiology

Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health

Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*

* Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and Institute for Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000 (received for review October 16, 2000)

Abstract
Top
Abstract
Introduction
Materials and Methods
Results
Discussion
Conclusions
References

There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.

Introduction

============================

IN light of Asante/Collinge et al findings that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

-------- Original Message --------

Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD

Date: Thu, 28 Nov 2002 10:23:43-0000

From: "Asante, Emmanuel A"

To:"'flounder@wt.net'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am
a Senior Scientist in the MRC Prion Unit and the lead author on the
paper. I have attached a pdf copy of the paper for your attention. Thank
you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you
will find in the paper, we have managed to associate the alternate
phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in
respect of Heidenhain variant CJD or Vicky Rimmer's version. It will
take further studies, which are on-going, to establish if there are
sub-types to our initial finding which we are now reporting. The main
point of the paper is that, as well as leading to the expected new
variant CJD phenotype, BSE transmission to the 129-methionine genotype
can lead to an alternate phenotype which is indistinguishable from type
2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I
can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial
College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG
Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:
e.asante@ic.ac.uk (until 9/12/02)
New e-mail: e.asante@prion.ucl.ac.uk (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

SCRAPIE USA MONTHLY REPORT 2005

AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure 3). There were 11 new infected and source flocks reported in March (Figure 4) with a total of 51 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 39 (Figure 6), with 1 flock released in March. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.76 : 1. IN addition, as of March 31, 2005, 225 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in March 2005 (Figure 8). Fourteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat cases was reported in January 2005. New infected flocks, source flocks, and flocks released or put on clean-up plans for FY 2005 are depicted in Figure 10. ...

FULL TEXT ;

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html

SCRAPIE USA JUNE 2005 UPDATE

AS of June 30, 2005, there were 114 scrapie infected and source flocks (Figure 3). There were 14 new infected and source flocks reported in June (Figure 4) with a total of 123 flocks reported for FY 2005 (Figure 5).

snip...

In addition, as of June 30, 2005, 448 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 106 were RSSS cases (Figure 7). This includes 81 newly confirmed cases in June 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005.

snip...end

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html

USA CWD MAP

http://www.aphis.usda.gov/vs/nahps/cwd/labmap.html

Perspective

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*

*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from:

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

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Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.

SNIP...

Conclusions

The lack of evidence of a link between CWD transmission and unusual cases of CJD, despite several epidemiologic investigations, and the absence of an increase in CJD incidence in Colorado and Wyoming suggest that the risk, if any, of transmission of CWD to humans is low. Although the in vitro studies indicating inefficient conversion of human prion protein by CWD-associated prions raise the possibility of low-level transmission of CWD to humans, no human cases of prion disease with strong evidence of a link with CWD have been identified. However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease. The level and frequency of human exposure to the CWD agent may increase with the spread of CWD in the United States. Because the number of studies seeking evidence for CWD transmission to humans is limited, more epidemiologic and laboratory studies should be conducted to monitor the possibility of such transmissions. Studies involving transgenic mice expressing human and cervid prion protein are in progress to further assess the potential for the CWD agent to cause human disease. Epidemiologic studies have also been initiated to identify human cases of prion disease among persons with an increased risk for exposure to potentially CWD-infected deer or elk meat (47). If such cases are identified, laboratory data showing similarities of the etiologic agent to that of the CWD agent would strengthen the conclusion for a causal link. Surveillance for human prion diseases, particularly in areas where CWD has been detected, remains important to effectively monitor the possible transmission of CWD to humans. Because of the long incubation period associated with prion diseases, convincing negative results from epidemiologic and experimental laboratory studies would likely require years of follow-up. In the meantime, to minimize the risk for exposure to the CWD agent, hunters should consult with their state wildlife agencies to identify areas where CWD occurs and continue to follow advice provided by public health and wildlife agencies. Hunters should avoid eating meat from deer and elk that look sick or test positive for CWD. They should wear gloves when field-dressing carcasses, bone-out the meat from the animal, and minimize handling of brain and spinal cord tissues. As a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified.

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Research

Environmental Sources of Prion Transmission in Mule Deer

Michael W. Miller,* Elizabeth S. Williams,† N. Thompson Hobbs,‡ and Lisa L. Wolfe*
*Colorado Division of Wildlife, Fort Collins, Colorado, USA; †University of Wyoming, Laramie, Wyoming, USA; and ‡Colorado State University, Fort Collins, Colorado, USA
Suggested citation for this article: Miller MW, Williams ES, Hobbs NT, Wolfe LL. Environmental sources of prion transmission in mule deer. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm

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Whether transmission of the chronic wasting disease (CWD) prion among cervids requires direct interaction with infected animals has been unclear. We report that CWD can be transmitted to susceptible animals indirectly, from environments contaminated by excreta or decomposed carcasses. Under experimental conditions, mule deer (Odocoileus hemionus) became infected in two of three paddocks containing naturally infected deer, in two of three paddocks where infected deer carcasses had decomposed in situ ≈1.8 years earlier, and in one of three paddocks where infected deer had last resided 2.2 years earlier. Indirect transmission and environmental persistence of infectious prions will complicate efforts to control CWD and perhaps other animal prion diseases.

snip...

Discussion

Prions cannot be directly demonstrated in excreta or soil. However, CWD infection–specific protease-resistant prion protein (PrPCWD) accumulates in gut-associated lymphoid tissues (e.g., tonsils, Peyer patches, and mesenteric lymph nodes) of infected mule deer (11,17,22), which implicates alimentary shedding of the CWD agent in both feces and saliva (10,11,17). Because PrPCWD becomes progressively abundant in nervous system and lymphoid tissues through the disease course (11), carcasses of deer succumbing to CWD also likely harbor considerable infectivity and thus serve as foci of infection. We could not determine the precise mechanism for CWD transmission in excreta-contaminated paddocks, but foraging and soil consumption seemed most plausible. Deer did not actively consume decomposed carcass remains, but they did forage in the immediate vicinity of carcass sites where a likely nutrient flush (23) produced lush vegetation (Figure).

Our findings show that environmental sources of infectivity may contribute to CWD epidemics and illustrate the potential complexity of such epidemics in natural populations. The relative importance of different routes of infection from the environment cannot be discerned from our experiment, but each could play a role in sustaining natural epidemics. Although confinement likely exaggerated transmission probabilities, conditions simulated by this experiment do arise in the wild. Mule deer live in established home ranges and show strong fidelity to historic home ranges (24-26). As a result of such behavior, encounters with contaminated environments will occur more frequently than if deer movements were random. Feces and carcass remains are routinely encountered on native ranges, thus representing natural opportunities for exposure. Social behavior of deer, particularly their tendency to concentrate and become sedentary on their winter range, also may increase the probability of coming into contact with sources of infection in their environment.

The ability of the CWD agent to persist in contaminated environments for >2 years may further increase the probability of transmission and protract epidemic dynamics (8). Because infectivity in contaminated paddocks could not be measured, neither the initial levels nor degradation rate of the CWD agent in the environment was estimable. However, the observed persistence of the CWD agent was comparable to that of the scrapie agent, which persisted in paddocks for ≈1 to 3 years after removal of naturally infected sheep (7). Similarities between the CWD and scrapie agents suggest that environmental persistence may be a common trait of prions. Whether persistence of the BSE prion in contaminated feed production facilities or in environments where cattle reside contributed to BSE cases in the United Kingdom after feed bans were enacted (27) remains uncertain but merits further consideration.

Indirect transmission and environmental persistence of prions will complicate efforts to control CWD and perhaps other animal prion diseases. Historically, control strategies for animal prion diseases have focused on infected live animals as the primary source of infection. Although live deer and elk represent the most plausible mechanism for geographic spread of CWD, our data show that environmental sources could contribute to maintaining and prolonging local epidemics, even when all infected animals are eliminated. Moreover, the efficacy of various culling strategies as control measures depends in part on the rates at which the CWD agent is added to and lost from the environment. Consequently, these dynamics and their implications for disease management need to be more completely understood.

snip...

http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm

For Release: Wednesday, May 4, 2005

Contact: Michael Fraser (518) 402-8000

DEC Announces Sampling Results for Chronic Wasting Disease

The New York State Department of Environmental Conservation today announced that it has received the remainder of test results for chronic wasting disease (CWD) that were part of intensive sampling efforts in central New York. DEC has received two positive results for the disease out of 292 wild deer sampled.

The first positive result in a wild deer was announced on April 27, 2005 and came from a yearling white-tailed deer sampled from the Town of Verona, Oneida County. The second positive result is from a 3-year-old doe, located within a mile of the location where the initial positive result was detected. The sample tissues were tested at the State's Veterinary Diagnostic Laboratory at Cornell University. These are the first known occurrences of CWD in wild deer in New York State.

DEC implemented intensive monitoring efforts after CWD was found in two captive white-tailed deer herds in Oneida County – the first incidents of CWD in New York State. On April 8, 2005, the State Department of Agriculture and Markets (DAM) completed testing of the captive deer and found a total of five positive results for CWD in the two captive herds.

DEC, along with the U.S. Department of Agriculture's Wildlife Services program, completed intensive monitoring on April 30, 2005. The effort resulted in 290 samples of wild deer from Oneida County, two from neighboring Madison County, and 25 wild deer from the Town of Arietta, Hamilton County. Since 2002, DEC has conducted statewide sampling of wild deer for CWD. When combined with sampling efforts in Oneida and Hamilton Counties, DEC has collected more than 3,700 samples from wild white-tailed deer.

DEC and DAM will continue public outreach to interested parties in central New York to help educate citizens on CWD and to discuss next steps to be taken. The agencies will hold a public meeting on Thursday, May 12, 2005, at 7 p.m. in the Vernon-Verona-Sherrill High School Auditorium, located on State Highway 31 in the Town of Verona. In addition, DEC and DAM will conduct additional outreach and continue to aggressively pursue inspection and enforcement across the State.

DAM continues to investigate, sample and test white-tailed deer from two captive herds directly associated with the two herds that were confirmed positive for CWD in Oneida County. Results for these sampling efforts will be announced when available.

Statewide sampling for CWD - which has resulted in more than 1,000 tests each year - will be increased to closely monitor the distribution and prevalence of CWD in wild deer. In addition, DEC has implemented emergency regulations regarding the handling, transport and management of deer in the State. The emergency regulations are currently in effect and represent an aggressive response to the recent discovery of chronic wasting disease (CWD) in Oneida County.

DEC's emergency regulations are designed to ensure the proper handling of deer and prevent further spread of CWD in the wild herd. The emergency regulations are effective for 90 days. In addition, DEC will begin the process of developing permanent regulations, which will appear in the State Register and include a 45-day public comment period.

CWD is a transmissible disease that affects the brain and central nervous system of certain deer and elk. There is no evidence that CWD is linked to disease in humans or domestic livestock other than deer and elk. More information on CWD can be found at DEC's website at

www.dec.state.ny.us/website/dfwmr/wildlife/deer/currentcwd.html

05-48

http://www.dec.state.ny.us/website/press/pressrel/2005/200548.html

WE MUST ADHERE TO THE BSE GBR RISK ASSESSMENTS, WE MUST WORK TO ENHANCE THOSE BSE GBR RISK ASSESSMENTS TO INCLUDE ALL ANIMAL TSEs, USDA/APHIS/GW ET ALs BSE MRR (Minimal Risk Region) should be REPEALED/DISBANDED/TRASHED/NADA and done away with for good. The BSE MRR policy is nothing more than a legal tool to trade all strains of TSEs globally...

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

Your Comment with Title "[Docket No. 05-004-1] RIN 0579-AB93 BSE TSS " was Received.
The Identifier Assigned is "APHIS-2005-0073-0009".

An Electronic File was Attached to this Submission.

Please note that it may take between 24 and 72 hours for the EDOCKET staff to process your comment before it is available publicly through EDOCKET. You can use the identifier noted above to find your comment through the Quick or Advanced Search pages when it is available.

TSS

U.S. Beef Talks May Progress as Japan Gathers Mad-Cow Disease Risk Data about U.S.A.

2010-10-15T12:42:00.000-07:00

U.S. Beef Talks May Progress as Japan Gathers Mad-Cow Disease Risk Data about U.S.A.

Greetings,

I thought i would add a bit of _data_, some of which probably is NOT in the information being _gathered_ by Japan, and given _to_ by the USDA et al $

U.S. Beef Talks May Progress as Japan Gathers Mad-Cow Disease Risk Data

By Aya Takada - Oct 14, 2010 9:53 PM CT

Japan, the biggest buyer of American beef before an outbreak of mad cow disease prompted the country to ban imports, is analyzing the safety of meat from cattle older than 20 months amid U.S. calls to normalize the trade.

Japan restricts U.S. beef imports to cattle aged 20 months or younger on concern that older animals may be at higher risk for the disease, also known as bovine spongiform encephalopathy. The nation’s Food Safety Commission must rule that any change in policy won’t increase human health risks, in order for imports of older cattle to resume.

Japan banned American beef when the U.S. disclosed its first case in 2003. The ban was relaxed in 2005 to allow meat from young cattle. Companies including Tyson Foods Inc. and Cargill Inc. lose about $1 billion in sales a year because of the restriction, said the National Cattlemen’s Beef Association.

“We have to collect enough data before submitting a request to the Food Safety Commission for risk assessment,” Minoru Yamamoto, director at the international animal health affairs office of the Ministry of Agriculture, Forestry and Fisheries, said in an interview in Tokyo. “We are seeking information from the U.S. and waiting for their replies.”

Mexico, South Korea and Taiwan buy U.S. beef from cattle younger than 30 months. Canada, Indonesia and Malaysia accept the meat from animals of any age, in line with international guidelines. Japan also requires U.S. shippers to remove risk materials, such as the spinal cord, that can transmit the disease to humans if consumed.

Beef Shipments

If Japan raises the age limit to 30 months, U.S. beef shipments to the Asian nation may return close to pre-ban levels, said Susumu Harada, senior director at the Tokyo office of the U.S. Meat Export Federation.

“The change would remove obstacles in the beef trade as U.S. products for overseas shipments are mostly from cattle aged up to 24 months,” Harada said in an interview.

Japan imported 475,000 metric tons of beef last fiscal year, of which 75 percent was from Australia and 16 percent was from the U.S., according to the agriculture ministry. In the year ended March 31, 2004, U.S. beef represented 201,000 tons, or 39 percent, of Japan’s total imports. Japan banned U.S. beef in December 2003, prompting restaurant chain operator Yoshinoya Holdings Co. to suspend sales of its “gyudon” beef bowl.

U.S. government representatives will visit Japan as Agriculture Minister Michihiko Kano will chair a ministerial meeting on food security of Asia-Pacific Economic Cooperation members from Oct. 16-17. Kano replaced Masahiko Yamada, known for his tough stance on food safety, when Prime Minister Naoto Kan reshuffled his cabinet on Sept. 17.

‘Sound Science’

U.S. lawmakers called President Barack Obama to address beef trade during his meeting with Kan when the Japanese prime minister visited the nation last month, saying Japan’s restrictions “are not based on sound science nor are they consistent with international guidelines.”

“At the very least, Japan should agree to immediately relax its age restrictions to 30 months as an interim step on a pathway that would amend its import protocol to be consistent with OIE guidelines,” senators including former Agriculture Secretary Mike Johanns and agriculture committee member Pat Roberts said in a letter to the president.

The World Organization for Animal Health, also known as OIE, voted in May 2007 to give the U.S. its “controlled-risk” rating for mad cow disease. The designation means controls are effective, and meat from U.S. cattle of any age can be safely traded. The OIE standards are used to settle trade disputes at the World Trade Organization.

Relaxing Restriction

Kano told reporters on Sept. 24 that Japan was “making a decision based on scientific knowledge” regarding food-safety issues. His remarks came a day after Foreign Minister Seiji Maehara was quoted by Kyodo News as telling Secretary of State Hillary Clinton that relaxing the age restriction would be considered.

Japan planned to ease the restriction on U.S. beef imports to allow meat from cattle aged up to 30 months in 2007 under the former government of the Liberal Democratic Party. The administration of President George W. Bush had urged Japan to eliminate the age limitation completely, in line with international standards, and the deadlock continued.

Japan and the U.S. held their first working-level meeting in three years last month, based on an agreement reached in April between Agriculture Secretary Tom Vilsack and then Agriculture Minister Hirotaka Akamatsu to resume beef talks.

Double Exports

President Obama’s administration increased pressure on Japan to re-open its market to U.S. beef as part of a goal to double the country’s exports in the next five years.

Japan was the third-largest destination for U.S. beef last year, at $470 million, up from $383 million in 2008, according to the U.S. Meat Export Federation. That compares with $1.391 billion in 2003. Mexico and Canada were the biggest buyers of U.S. beef last year.

More than 100 countries buy beef from the U.S., which has found three cases of the disease in the past two decades, and no incidents in the last three years, Vilsack said on April 9 in Tokyo.

To contact the reporter on this story: Aya Takada in Tokyo atakada2@bloomberg.net

To contact the editor responsible for this story: Richard Dobson at rdobson4@bloomberg.net

http://www.bloomberg.com/news/2010-10-15/u-s-beef-talks-may-progress-as-japan-gathers-data-on-mad-cow-disease-risk.html

=============================================

The World Organization for Animal Health, also known as OIE, voted in May 2007 to give the U.S. its “controlled-risk” rating for mad cow disease. The designation means controls are effective, and meat from U.S. cattle of any age can be safely traded. The OIE standards are used to settle trade disputes at the World Trade Organization.

=============================================

INDEED, thanks to the O.I.E., and the U.S.D.A. SSS policy, and the fact they are using science dated back to 1985 still in some cases. THE infamous June 2004 enhanced bse surveillance program was set up to fail from the beginning, and fail they did, not only with the surveillance for TSE, but also the partial and voluntary feed ban of August 4, 1997 was nothing but ink on paper. What i predicted a decade ago, has in fact come to pass, the TSE agent has mutated in every species from CWD now at two documented strains, BSE with 4 strains documented to date (c-BSE, h-BSE [typical h-BSE or the one and only documented g-h-BSEalabama strain?], l-BSE, and the IBNC BSE), and the different Scrapie strains are too many to count, not included the atypical Nor-98 and or BSE in sheep. with the 3 strains of BSE documented in North America to date, the two strains of CWD, TME, and all the strains of Scrapie, with 5 cases of the Nor-98 atypical scrapie cases already documented in 2010 here in the USA, all of which of the past two decades have been rendered and fed to food producing animals for animals and humans, and any human TSE there from ??? please note that the sporadic CJD case here in the USA has had a steady increase since 1997. it's not rocket science. what the USDA, FDA et al have used is junk science, bought and paid for by your local cattle dealer i.e. INDUSTRY. the BSE MRR policy was nothing more than a legal tool to do the same thing when the U.K. poisoned the globe with BSE, except now it's legal $$$

these are the facts as i have come to know them. just my take. ...

Let's take a look at the facts shall we $

BOVINE SPONGIFORM ENCEPHALOPATHY, SCRAPIE, CWD, CJD, NORTH AMERICA TYPICAL AND ATYPICAL

Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

Vet Pathol 0300985810382672, first published on October 4, 2010

Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

H. Okada okadahi@affrc.go.jp Prion Disease Research Center, National Institute of Animal Health, Tsukuba, K. Masujin Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Y. Imamaru Prion Disease Research Center, National Institute of Animal Health, Tsukuba, M. Imamura Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Y. Matsuura Prion Disease Research Center, National Institute of Animal Health, Tsukuba, S. Mohri Prion Disease Research Center, National Institute of Animal Health, Tsukuba, S. Czub Animal Disease Research Institute, Canadian Food Inspection Agency, T. Yokoyama Prion Disease Research Center, National Institute of Animal Health, Tsukuba,

Abstract

To characterize the biological and biochemical properties of H-type bovine spongiform encephalopathy (BSE), a transmission study with a Canadian H-type isolate was performed with bovinized transgenic mice (TgBoPrP), which were inoculated intracerebrally with brain homogenate from cattle with H-type BSE. All mice exhibited characteristic neurologic signs, and the subsequent passage showed a shortened incubation period. The distribution of disease-associated prion protein (PrPSc) was determined by immunohistochemistry, Western blot, and paraffin-embedded tissue (PET) blot. Biochemical properties and higher molecular weight of the glycoform pattern were well conserved within mice. Immunolabeled granular PrPSc, aggregates, and/or plaque-like deposits were mainly detected in the following brain locations: septal nuclei, subcallosal regions, hypothalamus, paraventricular nucleus of the thalamus, interstitial nucleus of the stria terminalis, and the reticular formation of the midbrain. Weak reactivity was detected by immunohistochemistry and PET blot in the cerebral cortex, most thalamic nuclei, the hippocampus, medulla oblongata, and cerebellum. These findings indicate that the H-type BSE prion has biological and biochemical properties distinct from those of C-type and L-type BSE in TgBoPrP mice, which suggests that TgBoPrP mice constitute a useful animal model to distinguish isolates from BSE-infected cattle.

© 2010 Sage Publications, Inc.

http://vet.sagepub.com/content/early/2010/10/02/0300985810382672.abstract

Greetings,

I have been most interested to see IF the h-BSE (h-BSE or g-h-BSEalabama???), but i have been most interested to see if in fact this atypical h-BSE is more virulent than c-BSE, as is the L-BSE (Italian strain) has been documented to be. We know from the studies of Kong et al that h-BSE will transmit to TG human mice;

BSE-H is also transmissible in our humanized Tg mice.

The possibility of more than two atypical BSE strains will be discussed.

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

HOWEVER, as to the virulance of it one way or the other compared to c-BSE and or L-BSE, i don't think no one has said yet or not? interesting this debate of the h-BSE TEXAS (2nd mad cow finally confirmed 7 months after the fact, and an act of Congress), compared to the g-h-BSEalabama strain documented in Alabama, that is identicle to the new human CJD in the USA that is killing the young and old, with clinical long duration, and different symptoms in some cases too, but not related to this ??? ALSO, this IBNC BSE, might this be the g-h-BSEalabama strain?

FOR anyone interested, please see my concerns here ;

Thursday, October 07, 2010

Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

http://bse-atypical.blogspot.com/2010/10/experimental-transmission-of-h-type.html

THIS is just ONE month report, of TWO recalls of prohibited banned MBM, which is illegal, mixed with 85% blood meal, which is still legal, but yet we know the TSE/BSE agent will transmit blood. we have this l-BSE in North America that is much more virulent and there is much concern with blood issue and l-BSE as there is with nvCJD in humans. some are even starting to be concerned with sporadic CJD and blood, and there are studies showing transmission there as well. ... this is one month recall page, where 10 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE, TO BE FED OUT. very little of the product that reaches commerce is ever returned via recall, very, very little. this was 2007, TEN YEARS AFTER THE AUGUST 4, 1997, PARTIAL AND VOLUNTARY MAD COW FEED BAN IN THE USA, that was nothing but ink on paper. i have listed the tonnage of mad cow feed that was in ALABAMA in one of the links too, this is where the infamous g-h-BSEalabama case was, a genetic relation matching the new sporadic CJD in the USA. seems this saga just keeps getting better and better.......$$$

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

Tuesday, March 2, 2010

Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA

http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html

Monday, March 1, 2010

ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010

http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html

Tuesday, September 14, 2010

Feed Safety and BSE/Ruminant Feed Ban Support Project (U18)

http://madcowfeed.blogspot.com/2010/09/feed-safety-and-bseruminant-feed-ban.html

Friday, October 8, 2010

Scientific reasons for a feed ban of meat-and-bone meal, applicable to all farmed animals including cattle, pigs, poultry, farmed fish and pet food

http://madcowfeed.blogspot.com/2010/10/scientific-reasons-for-feed-ban-of-meat.html

Date: June 21, 2007 at 2:49 pm PST

Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program

An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.

4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half

http://www.usda.gov/oig/webdocs/sarc070619.pdf

Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program Â Phase II and Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything they did before 2005 suspect," Brown said.

http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r

LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.

This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$

ALABAMA MAD COW g-h-BSEalabama

In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156

http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html

Discussion:

The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. It also suggests a similar cause or source for atypical BSE in these countries.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

REPORT OF THE WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY 1989

snip...

4.2.9 ...

Also, if it resulted from a localised chance transmission of the scrapie strain from sheep to cattle giving rise to a mutant, a different pattern of disease would have been expected: its range would have increased with time. Thus the evidence from Britain is against the disease being due to a new strain of the agent, but we note that in the United States from 1984 to 1988 outbreaks of scrapie in sheep flocks are reported to have Increased markedly, now being nearly 3 times as high as during any previous period (18).

http://collections.europarchive.org/tna/20080102132706/http://www.bseinquiry.gov.uk/files/ib/ibd1/tab02.pdf

http://collections.europarchive.org/tna/20080102193106/http://www.bseinquiry.gov.uk/files/mb/m12/tab12.pdf

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

" Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle."

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas. She left 6 Kids and a Husband. The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-

Physician Discharge Summary, Parkland Hospital, Dallas Texas

Admit Date: 12/29/2009

Discharge Date: 1/20/2010

Attending Provider: Greenberg, Benjamin Morris;

General Neurology Team: General Neurology Team

Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence. She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically.

Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.

http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8

Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed .

The key word here is diverse. What does diverse mean?

If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

SEE FULL TEXT ;

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101

.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

32 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606-12

33 YB88/10.00/1.1

http://web.archive.org/web/20040823105233/www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

SEE where sporadic cjd in the USA went from 59 cases in 1997, to 216 cases in 2009. a steady increase since 1997. ...TSS

http://www.cjdsurveillance.com/pdf/case-table.pdf

see full text ;

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

Discussion:

The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. It also suggests a similar cause or source for atypical BSE in these countries.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

Friday, August 27, 2010

NEW ATYPICAL NOR-98 SCRAPIE CASE DETECTED IDAHO NOW 5 CASES DOCUMENTED 2010

http://nor-98.blogspot.com/2010/08/new-atypical-nor-98-scrapie-case.html

Friday, September 24, 2010 BSE

Surveillance Continues to Benefit Canadian Cattle Producers September 24, 2010 - Notice to Industry

http://madcowtesting.blogspot.com/2010/09/bse-surveillance-continues-to-benefit.html

Saturday, October 2, 2010

BSE surveillance front and centre: CFIA and USA

http://madcowtesting.blogspot.com/2010/10/bse-surveillance-front-and-centre-cfia.html

PLEASE NOTE *

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Atypical BSE in Cattle

BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Responsibilities include:

Driving research at the National and OIE BSE reference lab to ensure project milestones are met successfully. Contributing to the preparation of project progress reports. Directing technical staff working on the project. Communicating and discussing results, progress and future direction with project principle investigator(s). Communicating with collaborative project partners. Qualifications:

Successful completion of a PhD degree in an area focusing on or related to prion diseases. Extensive experience with molecular and/or morphologic techniques used in studying prion diseases and/or other protein misfolding disorders. Ability to think independently and contribute new ideas. Excellent written and oral communication skills. Ability to multitask, prioritize, and meet challenges in a timely manner. Proficiency with Microsoft Office, especially Word, PowerPoint and Excel. How to apply:

Please send your application and/or inquiry to: Dr. Stefanie Czub, DVM, Ph.D. Head, National and OIE BSE Reference Laboratory Canadian Food Inspection Agency Lethbridge Laboratory P.O. Box 640, Township Road 9-1 Lethbridge, AB, T1J 3Z4 Canada

phone: +1-403-382-5500 +1-403-382-5500 ext. 5549 email: stefanie.czub@inspection.gc.ca

Contact Info:

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

Wednesday, September 08, 2010

CWD PRION CONGRESS SEPTEMBER 8-11 2010

http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html

Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html

Thursday, August 12, 2010

Seven main threats for the future linked to prions

http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html

http://prionpathy.blogspot.com/

When the OIE and the USDA et al collaborated to make legal the trading of Transmissible Spongiform Encephalopathy, when they did away with the BSE GBR risk assessments, where the USA, Canada, and Mexico were categorized as BSE GBR III. please see ;

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1211902594180.htm

Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the Geographical BSE Risk of USA

please see full text ;

http://www.efsa.europa.eu/en/scdocs/doc/3rax1.pdf

YET, in 2010, tons and tons of banned mad cow protein are still in commerce here in the USA, scientific studies are being misconstrued and manipulated by ARS USDA, which are still going by TSE science that is decades old, while refusing to acknowledge new scientific studies, and FOIA requests are still being held up by the USDA et al on these urgent matters (see source related materials below). CJD of unknown phenotype, in victims that are getting younger, with longer clinical course from first onset of symptoms to death are occurring, in fact, sporadic CJD is still rising, where the TSEs in the different species are mutating here in the USA, and we still have this same dog and pony show by the OIE and USDA et al. IF you go back and look at the Countries that went by these OIE BSE guidelines, most all came down with BSE. I have said it before, I was say it again now, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...TSS

Saturday, June 19, 2010

U.S. DENIED UPGRADED BSE STATUS FROM OIE

http://www.oie.int/eng/session2010/PDF%20Press%20releases/PRESS78_EN.pdf

see full text and reasons why here ;

http://usdameatexport.blogspot.com/2010/06/us-denied-upgraded-bse-status-from-oie.html

Saturday, April 10, 2010

TOYOTA VS MAD COW DISEASE USA OIE BSE MRR IMPORT AND EXPORT TRADE WARS

http://usdameatexport.blogspot.com/2010/04/toyota-vs-mad-cow-disease-usa-oie-bse.html

Friday, August 20, 2010

USDA: Animal Disease Traceability August 2010

http://naiscoolyes.blogspot.com/2010/08/usda-animal-disease-traceability-august.html

Docket APHIS-2007-0033 Docket Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Docket Type Rulemaking Document APHIS-2007-0033-0001 Document Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Public Submission APHIS-2007-0033-0002.1 Public Submission Title Attachment to Singeltary comment

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=090000648027c28e

http://www.regulations.gov/search/Regs/contentStreamer?objectId=090000648027c28e&disposition=attachment&contentType=pdf

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of 2002; [TSS SUBMISSION ON POTENTIAL FOR BSE/TSE & FMD 'SUITCASE BOMBS'] - TSS 1/27/03 (0)

Docket Management

Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305 Comment Number: EC-254 [TSS SUBMISSION]

http://www.aphis.usda.gov/vs/ceah/cei/bse_greece0701.htm

Greetings list members,

i just cannot accept this;

23 kg of meat in a suitcase (suitcase bomb...TSS)

The data do not provide a species of origin code for these

products, therefore they may not contain any ruminant product.

what kind of statement is this? how stupid do they think we are? it could also very well mean that _all_ of it was ruminant based products !

Terry S. Singeltary Sr., Bacliff, Texas USA

What is the level of passenger traffic arriving in the United States from Slovenia?

There were no direct flights from Slovenia to the US in fiscal year 2000.

APHIS-PPQ’s agriculture quarantine inspection monitoring sampled 27 air passengers from Slovenia for items of agricultural interest in fiscal year 2000.

One of these 27 passengers was carrying two kilograms of a meat item that could potentially harbor pathogens that cause BSE. This passenger arrived to Elizabeth, New York, in June 2000 and declared no intention to visit a farm or ranch in the US.

Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base http://www.aphis.usda.gov/vs/ceah/cei/bse_slovenia1101.htm

What is the level of passenger traffic arriving in the United States from the affected country?

A total of 45,438 passengers arrived in the US on direct flights from the Czech Republic in fiscal year 2000. It is likely that additional passengers originating in the Czech Republic traveled to the US on non-direct flights.

As part of APHIS-PPQ’s Agriculture Quarantine Inspection Monitoring, 238 air passengers from the Czech Republic were inspected for items of agricultural interest in fiscal year 2000. Of these, 10, or 4.2%, were found to be carrying a total of 17 kg of items that could potentially present a risk for BSE. None of the passengers with items reported plans to visit or work on a farm or ranch while in the US.

Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base http://www.aphis.usda.gov/vs/ceah/cei/bse_cz0601.htm

What are the US imports of affected animals or animal products from Austria?

Between 1998 and June 2001, US imports from Austria included goat meat, animal feeds, and sausage. The sausage and animals feeds were from unspecified species. Source: World Trade Atlas

snip...

What is the level of passenger traffic arriving in the United States from Austria?

A total of 168,598 passengers on direct flights from Austria arrived at US airports in fiscal year 2000.

An undetermined number of passengers from Austria arrived in the US via indirect flights. Under APHIS-PPQ’s agricultural quarantine inspection monitoring, 565 air passengers from Austria were sampled for items of agricultural interest in fiscal year 2000. Ten (10) of these passengers, or 1.7 percent, carried a total of 23 kg meat (non-pork) items that could potentially harbor the pathogen(s) that cause BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the US.

Source: US Dept. of Transportation; APHIS-PPQ http://www.aphis.usda.gov/vs/ceah/cei/bse_austria1201.htm

Greetings FDA and public,

if you go to the below site, and search all BSE known countries and check out their air traffic illegal meat they have confiscated, and check out the low number checked, compared to actual passenger traffic, would not take too much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.

[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.]]

if they were to have questioned the terrorist that bombed the Twin Towers with jets, if they were to have questioned them at flight school in the USA, i am sure that they would have said they did not intend to visit the Twin Towers as a flying bomb either. what am i thinking, they probably did ask this? stupid me.

http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01

http://www.regulations.gov/search/Regs/home.html#documentDetail?R=09000064801f8151

http://www.regulations.gov/search/Regs/contentStreamer?objectId=09000064801f8152&disposition=attachment&contentType=msw8

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028 Public Submission Title Comment from Terry S Singletary

Comment 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.

MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure. ...

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8151

your only fooling yourselves with this stupid ukbsenvcjd only theory, and the BSE methology of the OIE. most any coutnry that went by those same OIE BSE guidelines all went down with BSE.

THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE.

AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

http://www.regulations.gov/search/Regs/contentStreamer?objectId=09000064801e47e1&disposition=attachment&contentType=xml

SEE where sporadic cjd in the USA went from 59 cases in 1997, to 216 cases in 2009. a steady increase since 1997. ...TSS

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1997 114 68 59 9 0 0

to

2009 425 259 216 43 0 0

http://www.cjdsurveillance.com/pdf/case-table.pdf

PLEASE SEE FULL TEXT HERE ;

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

***+++***

Thursday, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 Friday, June 20, 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

layperson

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net

Netherlands reports 2nd BSE case this year 14 October 2010

2010-10-14T17:04:00.000-07:00

Netherlands reports 2nd BSE case this year

14 October 2010

THE HAGUE (BNO NEWS) -- A 13-year-old cow in the Netherlands on Thursday was found to be infected with BSE, which is commonly known as 'mad-cow disease', according to the country's Ministry of Agriculture, Nature and Food Quality.

Thursday's case is the second case of BSE in the Netherlands since May 2008. The other case was reported on September 3 when a cow was diagnosed with BSE at a farm in Tilburg, near the border with Belgium.

The ministry on Thursday said the cow had died on a farm in country, but did not reveal where the farm was located. "We can expect to see several more BSE-cases during the next few years," the ministry said in a statement.

This is because a number of cows are still alive from before a European Union feed ban went into effect on January 1, 2001. That ban prohibits certain animal products from being fed to cows, which can lead to BSE being spread among cows.

The Netherlands tested around 405,000 cattle in 2009, while around 7.5 million cattle were tested throughout the European Union. A total of 67 BSE cases were found, all of which were outside of the Netherlands.

The ministry said there is no need to take action because cows cannot infect each other with BSE. "But, as usual, the animals who were born on the same farm around the same time as the infected animal or ate the same food will be examined, as well as their offspring."

(Copyright 2010 by BNO News B.V. All rights reserved. Info: sales@bnonews.com.)

http://channel6newsonline.com/2010/10/netherlands-reports-2nd-bse-case-this-year/

PLEASE SEE FULL REPORT HERE ;

http://www.oie.int/wahis/public.php?page=single_report&pop=1&reportid=9852

also please see ;

PPo2-6:

Molecular Characterisation of Seven Austrian BSE Isolates: Two L-Type Cases and a Special C-Type Case

Jan P.M. Langeveld,1 Jo H.F. Erkens,1 Jorg G. Jacobs,1 Alex Bossers,1 Ines Rammel2 and Hermann Schildorfer2 1Central Veterinary Institute of Wageningen UR; Lelystad, The Netherlands; 2Austrian Agency for Health and Food Safety; Mödling, Austria

Key words: BSE, prion, diagnosis, strain type, subtype

Common bovine spongiform encephalopathy (C-type BSE) has been accounted for spread of the BSE-epidemic from UK to other countries. Rare aberrant BSE types H and L have been found in animals aging eight years and more. In Austria only seven cases of BSE have been detected. Two were from six year old animals, the others were derived from animals above age ten. Therefore, the potential presence of aberrant types was investigated. Similar methods as before published by Jacobs et al. (J Clin Microbiol 2007; 45:1821) were used; PrPres analysis was performed on western blots using group A, B and C antibodies as well as investigation of the susceptibility for digestion with proteinase K (PK). Five cases behaved as C-type, while two cases had typical properties of L-type BSE or BASE. The ages of these two animals were 11 and 12.5 yr. One of the C-type cases (age 12.5 yr) behaved peculiar in the way that its binding to antibodies 12B2 and P4 was relatively strong compared to that of core antibody L42. Finding all three different BSE types in cattle is most probable in cattle of 10 years and older. The fact that types L and H are also found in low incidence countries like Austria, Sweden, USA, Japan and Canada is indicative for natural presence of sporadic BSE in different forms. Safeguarding the food chain for BSE is only sensible when the old age animals are included in active monitoring (Funders: Dutch LNV ministry, Austrian AGES).

http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

10. 09. 10. - 12:00

Dead cow had BSE, test shows Austria has been hit by the eighth case of mad-cow disease (BSE) this year.

The federal health ministry announced today (Fri) post mortem tests have shown that a 15-year-old cow which died of old age on a farm in the Lower Austrian district of Wiener Neustadt had the disease.

The ministry, headed by Social Democrat (SPÖ) Alois Stöger, stressed customers’ health had not been endangered since none of the animal's meat had gone on sale.

Meanwhile, People’s Party (ÖVP) Environment Minister Nikolaus Berlakovich unveiled plans to set up a country-wide egg-farming database.

Berlakovich’s initiative comes after it emerged that a Styrian firm imported substandard eggs from Poland and Hungary to sell them as local products.

The minister said he thought of the already existing anti-BSE database as an example for the planned egg-trading platform.

Berlakovich said all traders and sellers of eggs will have to inform authorities about their business activities by entering sale figures and other information in the database.

http://austrianindependent.com/news/General_News/2010-09-10/4529/Dead_cow_had_BSE,_test_shows

Pressemeldungen 09.09.2010 15:37 Gesundheitsministerium bestätigt 8. BSE-Fall in Österreich Wien (BGF) - Am Dienstag, den 7. September 2010, wurde ein 1995 geborenes Rind aus Niederösterreich im Zuge der Untersuchung von sogenannten Risikotieren im Rahmen eines Schnelltests positiv auf BSE getestet. Das Untersuchungsergebnis wurde heute, Donnerstag, den 9. September, durch das nationale Referenzlabor der Österreichischen Agentur für Gesundheit und Ernährungssicherheit (AGES) in Mödling endgültig bestätigt. Da das Tier bereits verendet war und eine Untersuchung in der Tierkörperverwertung veranlasst wurde, bestand keine Gefahr, dass Fleisch in die Lebensmittelkette gelangte. Es handelt sich um den insgesamt achten BSE-Fall in Österreich.

Die Veterinärbehörde Niederösterreich hat in Abstimmung mit dem Bundesministerium für Gesundheit unverzüglich alle notwendigen Vorkehrungen getroffen. "Durch die gute Zusammenarbeit zwischen den Bundes- und Landesbehörden, bestand nie ein Risiko für die Konsumentinnen und Konsumenten. Das zeigt auch, dass die Maßnahmen zur Bekämpfung von BSE und zur Absicherung des Konsumentenschutzes greifen", betonte Gesundheitsminister Alois Stöger. Weitere Informationen zum Thema BSE: http://www.bmg.gv.at Rückfragehinweis: Bundesministerium für Gesundheit Fabian Fußeis, Pressesprecher Radetzkystraße 2, 1030 Wien Tel.: +43/1/71100-4505 , Fax: +43/1/71100-14304 mailto:fabian.fusseis@bmg.gv.at

http://www.bmg.gv.at Digitale Pressemappe:

http://www.ots.at/pressemappe/52/aom

*** OTS-ORIGINALTEXT PRESSEAUSSENDUNG UNTER AUSSCHLIESSLICHER INHALTLICHER VERANTWORTUNG DES AUSSENDERS - WWW.OTS.AT *** OTS0284 2010-09-09/15:37

http://www.bmg.gv.at/cms/site/presse_detail.html?channel=CH0616&doc=CMS1284039658617

News Am Dienstag, den 7. September 2010, wurde ein 1995 geborenes Rind aus Niederösterreich im Zuge der Untersuchung von sogenannten Risikotieren im Rahmen eines Schnelltests positiv auf BSE getestet. Das Untersuchungsergebnis wurde am Donnerstag, den 09. September, durch das nationale Referenzlabor der Österreichischen Agentur für Gesundheit und Ernährungssicherheit (AGES) in Mödling endgültig bestätigt. Da das Tier bereits verendet war und eine Untersuchung in der Tierkörperverwertung veranlasst wurde, bestand keine Gefahr, dass Fleisch in die Lebensmittelkette gelangte. Es handelt sich um den insgesamt achten BSE-Fall in Österreich. Die Veterinärbehörde Niederösterreich hat in Abstimmung mit dem Bundesministerium für Gesundheit unverzüglich alle notwendigen Vorkehrungen getroffen. „Durch die gute Zusammenarbeit zwischen den Bundes- und Landesbehörden, bestand nie ein Risiko für die Konsumentinnen und Konsumenten. Das zeigt auch, dass die Maßnahmen zur Bekämpfung von BSE und zur Absicherung des Konsumentenschutzes greifen“, betonte Gesundheitsminister Alois Stöger.

Aktuelle Informationen BSE (DOC 42 KB) 9.09.2010 - 8. Fall bestätigt

http://www.bmg.gv.at/cms/site/news_detail.html?channel=CH0518&doc=CMS1284038655071

==========================

Thursday, October 07, 2010

Experimental Transmission of H-type Bovine Spongiform Encephalopathy to Bovinized Transgenic Mice

http://bse-atypical.blogspot.com/2010/10/experimental-transmission-of-h-type.html

Saturday, October 2, 2010

BSE surveillance front and centre: CFIA and USA

http://madcowtesting.blogspot.com/2010/10/bse-surveillance-front-and-centre-cfia.html

Tuesday, September 28, 2010

Variant CJD: where has it gone, or has it?

Pract Neurol 2010; 10: 250–251

http://vcjdtransfusion.blogspot.com/2010/09/variant-cjd-where-has-it-gone-or-has-it.html

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

THIS is why this question is so important to me, IS h-BSE more or less virulent than the c-BSE ?

Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8

Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed .

The key word here is diverse. What does diverse mean?

If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

SEE FULL TEXT ;

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101

.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

32 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606-12

33 YB88/10.00/1.1

http://web.archive.org/web/20040823105233/www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. It also suggests a similar cause or source for atypical BSE in these countries.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

REPORT OF THE WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY 1989

snip...

4.2.9 ...Also, if it resulted from a localised chance transmission of the scrapie strain from sheep to cattle giving rise to a mutant, a different pattern of disease would have been expected: its range would have increased with time. Thus the evidence from Britain is against the disease being due to a new strain of the agent, but we note that in the United States from 1984 to 1988 outbreaks of scrapie in sheep flocks are reported to have Increased markedly, now being nearly 3 times as high as during any previous period (18).

http://collections.europarchive.org/tna/20080102132706/http://www.bseinquiry.gov.uk/files/ib/ibd1/tab02.pdf

http://collections.europarchive.org/tna/20080102193106/http://www.bseinquiry.gov.uk/files/mb/m12/tab12.pdf

THIS is just ONE month report, of TWO recalls of prohibited banned MBM, which is illegal, mixed with 85% blood meal, which is still legal, but yet we know the TSE/BSE agent will transmit blood. we have this l-BSE in North America that is much more virulent and there is much concern with blood issue and l-BSE as there is with nvCJD in humans. some are even starting to be concerned with sporadic CJD and blood, and there are studies showing transmission there as well. ... this is one month recall page, where 10 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE, TO BE FED OUT. very little of the product that reaches commerce is ever returned via recall, very, very little. this was 2007, TEN YEARS AFTER THE AUGUST 4, 1997, PARTIAL AND VOLUNTARY MAD COW FEED BAN IN THE USA, that was nothing but ink on paper. i have listed the tonnage of mad cow feed that was in ALABAMA in one of the links too, this is where the infamous g-h-BSEalabama case was, a genetic relation matching the new sporadic CJD in the USA. seems this saga just keeps getting better and better.......$$$

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

Tuesday, September 14, 2010

Feed Safety and BSE/Ruminant Feed Ban Support Project (U18)

http://madcowfeed.blogspot.com/2010/09/feed-safety-and-bseruminant-feed-ban.html

Saturday, October 2, 2010

BSE surveillance front and centre: CFIA and USA

http://madcowtesting.blogspot.com/2010/10/bse-surveillance-front-and-centre-cfia.html

SEE where sporadic cjd in the USA went from 59 cases in 1997, to 216 cases in 2009. a steady increase since 1997. ...TSS

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1997 114 68 59 9 0 0

to

2009 425 259 216 43 0 0

http://www.cjdsurveillance.com/pdf/case-table.pdf

PLEASE SEE FULL TEXT HERE ;

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

***+++***

Thursday, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 Friday, June 20, 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

TSS

COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010

2010-03-16T13:29:00.000-07:00

AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard

SENATE RURAL AND REGIONAL AFFAIRS AND TRANSPORT REFERENCES COMMITTEE Reference: Import restrictions on beef

FRIDAY, 5 FEBRUARY 2010 CANBERRA CONDITIONS OF DISTRIBUTION

This is an uncorrected proof of evidence taken before the committee. It is made available under the condition that it is recognised as such. BY AUTHORITY OF THE SENATE [PROOF COPY] TO EXPEDITE DELIVERY, THIS TRANSCRIPT HAS NOT BEEN SUBEDITED

SNIP...

Friday, 5 February 2010 Senate RRA&T 1

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Committee met at 9.01 am

CHAIR (Senator Nash)—I declare open this public hearing of the Rural and Regional Affairs and Transport References Committee. The committee is hearing evidence on the committee’s inquiry into the impact and consequences of the government’s decision to relax import restrictions on beef. Before the committee starts taking evidence I remind all witnesses that, in giving evidence to the committee, they are protected by parliamentary privilege. It is unlawful for anyone to threaten or disadvantage a witness on account of evidence given to a committee and such action may be treated by the Senate as a contempt. It is also a contempt to give false or misleading evidence to a committee. The committee prefers all evidence to be given in public but, under the Senate’s resolutions, witnesses have the right to request to be heard in private session. It is important that witnesses give the committee notice if they intend to ask to give evidence in camera. If a witness objects to answering a question, the witness should state the ground upon which the objection is taken and the committee will determine whether it will insist on an answer, having regard to the ground which is claimed. If the committee determines to insist on an answer, a witness may request that the answer be given in camera. Such a request may, of course, also been made at any other time. On behalf of the committee, I thank all those who have made submissions and sent representatives here today for their cooperation in this inquiry.

RRA&T 2 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

[9.03 am]

BELLINGER, Mr Brad, Chairman, Australian Beef Association

CARTER, Mr John Edward, Director, Australian Beef Association

CHAIR—Welcome. Would you like to make an opening statement?

Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so:

You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heinemann variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:

The OIE— the International Organisation of Epizootics, the arm of the WTO— is a failed global agent that in my opinion is bought off via bogus regulations for global trade and industry reps. I have done this all these years for nothing but the truth. I am a consumer, I eat meat, but I do not have to sit idly by and see the ignorance and greed of it all while countless numbers of humans and animals are being exposed to the TSE agents. All the USA is interested in is trade, nothing else matters.

Even Dr Stanley Prusiner, who incidentally won the Nobel Health Prize in 1997 for his work on the prion—he invented the word ‘prion’, or it came from him—states:

The BSC policy was set up for one purpose only, trade—the illegal trading of all strains of TSE globally throughout North America, which is home to CBSC, IBSC and HBSC, many scrapie strains and two strains of CJD to date. (please note typo error, those should have read cBSE, lBSE, and hBSE...tss)

I would also like, while I have the opportunity, to explain the beef-off-the-shelves myth. At the first Senate hearing on 14 December, it was explained that the reason why they allowed BSC beef into Australia was the beef-off-the-shelves policy, whereby if we found a case of BSC in Australia they would have to recall all—

Friday, 5 February 2010 Senate RRA&T 3

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Senator HEFFERNAN—Which of course is total BS.

Mr Bellinger—Correct. This is written in the FSANZ document—Food Standards Australia New Zealand. Why isn’t this same policy in New Zealand? It is not—it is only in Australia. We are the only country in the world to have this idiotic policy. So we again call for the tabling of the WTO obligations paperwork. We do not believe that exists.

Mr Carter—We have an additional concern about human health. We are not scientists, but on 18 December, four days after the last hearing here, the BBC reported a new wave of deaths due to variant CJD linked to eating BSE infected beef could be underway. This is based on the work of Professor John Collinge of the National Prion Clinic, who reported that a 2009 death in Scotland was from a different genetic pool to that of the 166 deaths already reported in the UK. Those are all thought to share one gene, but Professor Collinge and his colleagues estimate that up to 350 people in this new group, represented by the person who died in Scotland, could get CJD. He thinks that CJD has moved into a new phase, and the incubation period is a long one. We tender the Australian Red Cross donor policy sheet, which bears out what Senator Back brought up last time, questioning the Chief Medical Officer, and we say that blood from people who were in the UK between 1980 and 1996 is not acceptable. That is the current ruling. We believe this now should be extended to anyone who has visited the UK, and this new evidence should ensure that Australia revisits the science of CJD.

CHAIR—Thank you, Mr Carter. Before we kick off, can I just remind colleagues that we are short of time today, so I ask that we do not traverse ground the we have previously covered and make sure that we stick to new information that is required. Mr Bellinger, when you started you referred to your view that this decision to allow the importation was politically based. I know you are going to go into this in the course of the next 20 minutes or so, but could you just give us a quick outline of what your definition of politically based is and why you think the decision was politically based?

Mr Bellinger—On the lowering of BSE standards: if you go back to 2006, for example, there were five categories for describing countries that had BSE and Australia was in the category for BSE free. Suddenly, by the time the United States got their third instance of BSE, through the influence of Robert Zoellick—who was the trade minister that signed the BSE corresponding side letter in 2004 and was George Bush’s appointment to the WTO—they suddenly changed the five categories to three categories and, instead of being BSE free, Australia became BSE negligible risk. At the time I put out a press release alerting the media to the dangers of this happening, and we are coming to the stage here when suddenly our government is saying, ‘Now let’s allow the importation of beef from BSE affected countries.’ I believe that the WTO has been influenced by large multinational meat processors and retailers to change and allow the trading of BSE beef throughout the world.

CHAIR—Thanks, Mr Bellinger.

Mr Carter—Of course, the side letter that Minister Vaile signed was at the request of Mr Zoellick, who is now in the position that Mr Bellinger has explained.

Senator HEFFERNAN—I just want to put the committee on notice that, if we do not get through what we have got to get through today, I suggest we have another hearing, because this

RRA&T 4 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

is the greatest ambush of Australia’s farmers of all time by a government. The evidence given at the last meeting was deadset lies. The proposition that this whole change of government policy was led by the industry is a deadset lie. While Simon Crean might want to change his mind because of the WTO and his lack of knowledge, the Australian beef industry, as you know, is under great challenge, not only from the currency but also from the undermining of our markets. This is a disgrace.

I will go to the meat-off-the-shelves proposition. By the way, there is no obligation to take meat off the shelves; it was something dreamt up by someone buried in the bureaucracy, who is probably taking notes down in the department now. There is absolutely no obligation but, if they wanted to stick to it, all they really had to do was do mandatory SRM removal. Now the renderers did not like that idea. It was going to cost money. Can you explain to us where you think the meat-off-the-shelves proposition came from?

Mr Bellinger—I think it was an ill-informed, misguided statement delivered from RMAC to the minister. They may have thought, by some weird dream, that by having this beef-off-theshelves policy it would somehow illustrate to the WTO that we cannot import beef from BSE affected countries—totally erroneous and totally stupid. Of course, if you look at the legislation on food recalls, it is handled by the states. I did an interview on 2NZ, a local radio station, 10 days ago in reply to Tony Burke’s statement on this beef-off-the-shelves policy, where he said that if a beast was found to have BSE in the Northern Territory then beef would have to be taken off the shelves in Tasmania—totally erroneous. It does not exist. It is the states who handle this. I am amazed that a minister could make this sort of statement.

Senator HEFFERNAN—You were talking about the next wave of possible human infection in the UK. It is a fact, actually—I have done some work on it. There are three genes that have been identified. One is very accepting: if you have that gene and you eat the meat, you get the consequences—mad cow disease. There are two genes that they have not worked out yet. One is more resistant than the other. Are you aware that there is a lot of science around that says, through this new understanding of the gene mutation, there could well be a new wave of mad cow disease in humans?

Mr Bellinger—I will hand you over to John Carter. He has done more research on this than me.

Mr Carter—It is Professor Collinge and the National Prion Clinic in the UK who have done this work. It is not as though it is some backyard person. I am certainly not a geneticist, but it appears to me, particularly from the work that Bob Steel has done, that these things are crossing barriers. To me, the science is not proven at all.

Senator HEFFERNAN—No. There seems to have been a change of government position in assessing the science, to risk analysis from a lesser proposition. Are you aware of when the government changed from the precautionary principle to risk analysis in terms of assessing these sorts of risk?

Mr Carter—To me, those are just words. In 1997 the UK government Lord Phillips inquiry stated that up to 136,000 people could lose their lives to CJD, and later the Blair government raised this to 250,000. Then, of course, when America gets BSE suddenly it is really no problem.

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Senator HEFFERNAN—Look, this is just a trade issue. The government came in here.

Senator Sterle, I believe, will make some reflection upon your earlier remarks to say that this—

Senator STERLE—Absolutely, if you give me a chance. The clock is ticking.

Senator HEFFERNAN—Would you like to do it now?

Senator STERLE—Finish your question. No, because you will interrupt. So you have your run and then I will have my say.

Senator HEFFERNAN—The proposition was put to us last time that this was driven by the industry. I followed Simon Crean on 5AA the other day. He talked about ‘using the best principles’. He had idea what he was talking about, but they did say that this was driven by the industry—and the department accepted it, and I hope they are all listening down there, because they are a bunch of liars. They accepted the proposition—

Senator STERLE—Chair—

Senator O’BRIEN—Using this hearing to slander people in that way is completely unseemly.

Senator HEFFERNAN—Righto, I withdraw that. They overlooked the facts. We will correct the facts today, but this was driven by the industry, by demands from the industry, when in fact we now know from in an in-confidence answer to this committee there were 37 communications between the Canadian and US governments and the Australian government since November 2007 demanding that we allow their meat in here. Of course, the department of trade here— Simon Crean’s mob—said, ‘We’d better find a way,’ and this is the way they have done it. They have completely misled the Australian beef industry and I think it is a total disgrace. I might add also that, back when we knocked this on my head—

CHAIR—Do you have any questions, Senator Heffernan?

Senator HEFFERNAN—in 2005, I think it was, the then shadow health minister, Julia Gillard, was keen on maintaining the precautionary principle. We now seem to have gone from the precautionary principle, without any mention—by the way, this is all going to happen without coming to parliament—of risk analysis. I am going to deal with the risk analysis proposition when we get the guy that gave the so-called independent advice along. I will leave it at that.

Senator STERLE—I just have to clarify a few things, Mr Bellinger. You said that the OIE is an arm of the WTO. Do you want to clarify that? I am led to believe that they are not.

Mr Bellinger—I will explain it further. You have the World Trade Organisation, and then under that you have the World Health Organisation. The OIE, the organisation of international epizootics, then handles phytosanitary and animal health issues under that umbrella.

Senator STERLE—So they are not an arm of the WTO? I want to clarify it for the record.

Mr Bellinger—They are an arm—

snip...

Senator STERLE—Let us get back to the law. Quite clearly, 152 WTO members have the policy that if there is an outbreak, regardless of where that outbreak is, all meat must be taken off the shelves. Whether that has happened in America or not, this is the previous government’s policy.

Senator HEFFERNAN—No, it is not. That is garbage.

snip...

Dr Clegg—Nothing. Until the assessments are complete, nothing does change.

Senator HEFFERNAN—With Brazil, the policy there was developed in 1999. We brought in those cartons of beef from Brazil. As Mr McCutcheon said it had this whoopee tick from OIE and that was the policy in 1999 and there was no trace-back mechanism. You mob are saying, ‘We don’t care if the country has no traceability because we are not interested in herd status or national status only beef status.’ So it could come from Mexico, across the border, into some abattoir in Texas.

With Brazil there was no trace-back mechanism at all. DAFF did not inspect any of the facilities and did not bother to go over there, let alone send out teams. You were going to after the event, until the cartons arrived. What is going to change? Aren’t we entitled, for God’s sake, to know what the bloody protocols are so we can test them? That is a bloody good example of an OIE certification that went badly wrong. How do we know it is not going to go wrong again, for God’s sake?

Dr Carroll—We are not using OIE certifications.

Senator HEFFERNAN—Mr McCutcheon just said that you were.

Dr Carroll—No, he said we are using an OIE process.

Senator HEFFERNAN—Do not give me that mumbo jumbo bureaucratic crap.

Dr Carroll—Sorry, Senator, but an OIE process is different to an OIE certification. Senator HEFFERNAN—You still have not reported back on Brazil and what went wrong there.

Dr Carroll—The OIE categorises countries.

snip...

Dr Steel—Absolutely. If you look at the American system, there is some interplay. The Canadians and the Mexicans are taking the Americans on in the WHO because of country-oforigin limitations, so they cannot import their beef. The United States Department of Agriculture was against the COOL decisions. Now they have totally changed around. Why have they changed? You do not have to be Einstein to realise it is because of the risk of BSE. Just getting back to the point about huge undeclared amounts: the analogy on that graph is from Professor Mathews. They are not required to state how many cases they have got; they are only required to say that they have got BSE.

Senator BACK—On another point that I will ask you to comment on as it seems ironic to me: you and I would recall that in the 1970s it was the US government that demanded that Australia be free of brucellosis, knowing very well that brucellosis never had any involvement with meat. Yet this country spent millions and millions of dollars becoming free of brucellosis, and that was what led initially to the herd identification schemes et cetera. Those were all demanded by the US government, not because of health issues but because of trade issues. They then demanded that we increase the quality of our export abattoirs to a level infinitely higher than existed elsewhere in the world, not because of any health issues but because of trade issues. Does it seem unusual to you, with the boot now being on the other foot because Australia and New Zealand are the only region that is free of this, that the demands are being made by those very same countries in reverse? Does that seem ironic to you?

Dr Steel—I do not know about that Senator, but I do know that I am extremely proud of what the departments of agriculture have done and their incredible schemes. Senator BACK—So am I. Some of us built our practices on them.

Dr Steel—It may be ironical, but I still think that we have every reason to laud the departments who look after us. Now, unfortunately, I feel that our departments are not looking after us. I feel, particularly because of the OIE science, and I have referred you to that, there are grey areas. There is one individual, who I will not mention, who I feel has not been appropriate for Australian agriculture.

Senator BACK—Thank you. I will stop it at that because others may have questions.

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Senator McGAURAN—Is that the minister you are talking about?

Senator STERLE—It might be you, Julian! Dr Steel, just very quickly, you referred to New Zealand in your opening statement, but New Zealand has the policy that the Australian government is trying to put in here. It is the same policy, and they have had it for eight years without any problems.

Dr Steel—They made the decision after the scrapie was there, I believe. They are in the soup, so what have they got to lose? They have lost that uniqueness that we both had of being free of any TSEs, officially anyway. What have they got to lose? And it improves their trade. Now, because of the new technological advances in detection of TSEs, BSE and scrapie, and because muscle tissue has been found to contain prions—misfolded prions—our government can no longer say, ‘We’re importing beef and it has got nothing in its muscles to suggest infection.’ If you have got BSE in undeclared or unknown numbers, in unidentified cattle, how could we possibly not have a risk? Does that answer your question?

Senator STERLE—Thanks, Dr Steel. I know time is of the essence here so I will hand back to the chair.

CHAIR—As there are no further questions, thank you very much for appearing here today Dr Steel. We do appreciate your giving us your time.

Proceedings suspended from 10.11 am to 10.33 am

snip...

Dr Lavender—I would have to say no. I know that in Japan they do test every carcass. Having been to Japan a few times, I think they probably would do that. I would not have absolute confidence here. In America, as I understand it, testing was at a higher level and has gradually been decreased because in their own meat they do not want to find it because it would impact on their markets in countries such as Japan.

CHAIR—That is a very interesting point.

snip...

Senator HEFFERNAN—All right, let us go to the risk. Do you accept that any increase in
the risk of contracting CJD is unacceptable?

Prof. Mathews—Hypothetical it is unacceptable. But we all accept risks every day. The one
substantial piece of feedback I got from the NHMRC committee, when they looked at my draft
report, was where I had written in my conclusions that I believed the risk to the Australian
population was negligible. They asked me to quantify the risk. Hence I revised the report to put
in the quantification comparing the risk of variant CJD in the Australian population with the risk
from motorcar accidents. As you see in the report, I conclude that it is orders of magnitude more
likely that anyone in Australia—

Senator HEFFERNAN—But what you are saying to me now is that it is acceptable to have
an increased risk because once we do this there is an increased risk. You are saying that is
acceptable.

Prof. Mathews—Of course.

Senator HEFFERNAN—That is all I want you to answer. You are happy to accept the risk.

Prof. Mathews—But you are not arguing that we ban motorcars, Senator, are you?

CHAIR—We have got enough to deal with with beef. I think we will leave cars to another
inquiry.

Senator HEFFERNAN—Do you support the proposition that importing beef products into
Australia from countries with a history of BSE overseas—given there is all sorts of work going
on and no live tests et cetera and there are a lot of likeable rogues in the beef industry—poses no,
and I mean absolutely no, increased risk to the Australian population?

Prof. Mathews—I am not really to comment on the political end of it.

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Senator HEFFERNAN—No, but as a scientist are you saying—and I will repeat it—that
importing beef products into Australia from countries with a history of BSE overseas poses no
increased risk to the Australian population?

Prof. Mathews—No. I am not saying that. I am saying that any increased risk would be
negligible and I tried to quantify how negligible in my report.

snip...

Senator HEFFERNAN—Yes, and obviously we will come to that, and Senator Back is anxious to get some questions in. But against the background, we know this is all just colour and movement for the trade position and the other countries wanting to undermine our clean, green and free status and get equal standing in the market. There is no question about that. I draw your attention to the development of new testing for BSE and CJD. Wouldn’t it be prudent for the Australian government to wait until these testing processes, or more comprehensive ones, were proven and available generally so we can provide certainty before lessening any of the protective controls, which is the ultimate control—that is, you cannot bring it in. Shouldn’t we wait?

Prof. Mathews—You are asking me to comment on economic and political issues.

snip...

Senator HEFFERNAN—All right, Dr Carroll. You are the genius on this. Why wouldn’t we have and demand full traceability if we are going to import meat from a country so that we know where it is coming from? I am talking about whole-of-life traceability. Not where it was killed but where it came from. Why wouldn’t we demand that?

Dr Carroll—Part of the FSANZ process will be around what measures are in place to ensure the beef comes from countries—

Senator HEFFERNAN—But aren’t we entitled to know that, God’s sake? This is many times bigger than the IRA that was remanding pork and you are saying, ‘Beef? Bugger off. We are not interested. We do not need an IRA.’ What an insult to Australia’s cattle farmers. What an insult. You are comfortable; I can see that. We are angry about this. You say we do not need an IRA. It is completely changed. We do not even know the science of the crossover of the wasting disease from deer.

Dr Carroll—There is no science on the crossover of the wasting disease from deer. Senator HEFFERNAN—We do not know, you see. We do not know. There is no need for an IRA you say?

Dr Carroll—Correct.

Senator HEFFERNAN—How do we know if someone says they are going to bring in meat from Canada that it does not come from somewhere else if there is no traceability? Dr Carroll—That will be part of the process that FSANZ will be—

Senator HEFFERNAN—Aren’t we entitled—

CHAIR—Hang on. Just let the witness answer.

Dr Carroll—That will be part of the process FSANZ will carry out in assessing the country to see if it is eligible for a category A or a category B status.

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Senator HEFFERNAN—But why, for God’s sake, can’t you at least set some standards that we can work on, or are you bureaucrats too scared to be human failure tested?

CHAIR—Senator Heffernan—

Senator HEFFERNAN—It is a fair thing to say. If they are not going to tell us until after the event and are saying, ‘We do not want you to test it. You are a farmer; you would not know,’ why wouldn’t we be entitled to say to other countries, ‘We demand full traceability as we have here.’ What is wrong with that?

Dr Carroll—We have full traceability here for a range of reasons that go far beyond BSE and far beyond various other—

CHAIR—If I am correct in distilling down through all of this to exactly what you mean, the only thing that has changed is that now more countries can use the same process that has already existed because of a policy change.

Dr Carroll—The previous policy position—

CHAIR—Can you just give me a yes or a no.

Dr Clegg—No.

CHAIR—Okay.

Dr Clegg—There is a policy already on the website today that describes the assessment for countries—the Australian BSE assessment committee. That is on the FSANZ website now. You can have a look at that. The one that comes into place from 1 March 2010 differs slightly. There are differences in country categorisations. In the current policy, countries are categorised as A, B, C or D. In the new policy they are categories 1 and 2, with category 3 not assessed or not permitted.

CHAIR—So the policy is just broadening out—sorry if I am being really simplistic here— those countries that are now eligible to send their beef to Australia.

Dr Clegg—No. It is to be considered by the assessment panel. It has to be considered. It is just the terms of reference under which they are considered that has altered slightly. That is all. CHAIR—I will put it differently. At the moment there are countries that are precluded on the basis that they have had BSE.

Dr Clegg—Yes.

CHAIR—The change is that it is now opened up and those countries have an opportunity to apply.

Dr Clegg—Correct.

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CHAIR—All right. That then goes back to—and I want to get to the process for assessment shortly—how important the protocols are if those countries that previously have had BSE are now going to have an opportunity that they previously did not.

Dr Clegg—Sure.

CHAIR—That is how important the protocols and there is no way to have any kind of accountability to determine what those protocols should be because it is all in the hands of FSANZ.

Dr Clegg—They are the risk assessors.

CHAIR—I understand they are the risk assessors, but there are an awful lot of people in this country who would be very pleased to be able to have input if they feel that those protocols are not correct. Who is actually drawing them up, Mr McCutcheon?

Mr McCutcheon—They are being drawn up by officers of FSANZ.

CHAIR—Which ones?

Mr McCutcheon—The team consists of Andrew Bartholomaeus—he is leading that team— Amanda Hill and Dr Scott Crerar, at this stage. We are also employing another three officers to be part of that team. The recruitment process is pretty much nearing its conclusion.

CHAIR—So there are three people and three assistants. Does that go to the board?

Mr McCutcheon—No, those sorts of things do not go to the board. As I indicated at the last hearing, this was an additional function provided to FSANZ and we were provided with additional money to do that. Essentially, we are tailoring our resources with that additional budgetary funding.

CHAIR—So there are essentially those three key people who are writing the protocols? Mr McCutcheon—That is right, but they are not starting from scratch, if I can just reassure you, Senator.

CHAIR—No, I understand all that, but, at the end of the day, somebody has to sit down with a bit of paper or in front of a keyboard and write down what they are going to be. What is the process once they have finished writing?

Mr McCutcheon—Once they have finished it, there will certainly be consultation within the Australian government as we finalise those. Then, as I mentioned earlier in the hearing today, we will be getting an independent look at that by an OIE appointed expert. That will be done. Once it passes that test, we can finalise that protocol and that will be the protocol upon which countries will be able to make their applications.

CHAIR—Which, as you have said before, you can change at any time—FSANZ.

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Mr McCutcheon—That is correct in theory, but it is not something that—

CHAIR—No, but I just want to know what is possible and what is not possible down the track. At the moment, what is the process for assessing a country’s risk status?

Dr Clegg—Risk status for BSE or for any beef—

CHAIR—For BSE.

Mr McCutcheon—At the moment, if a country has had a BSE case, then it is not eligible to have an assessment done.

CHAIR—So it is as simple as that.

Mr McCutcheon—That is right.

CHAIR—What happens from 1 March?

Mr McCutcheon—For those countries that have a history of BSE, they are eligible to lodge an application to have their categorisation re-examined.

CHAIR—Who is going to do that?

Mr McCutcheon—That would be the Australian BSE safety assessment committee, which is essentially a FSANZ committee with one outsider from Biosecurity Australia.

Senator HEFFERNAN—Where will they get their advice from?

Mr McCutcheon—Much of the information we hope will be contained in the applications that come in from countries that lodge applications. Again, we have access to a whole range of experts within and outside Australia, so if we need additional information we will consult with those experts.

CHAIR—Could you just clarify for me exactly the change in policy? There is nothing in regulation, there is nothing in legislation, so this is just something that happens within the department or departments—I am not quite sure where. Perhaps you could outline for me very clearly how the policy change decision manifests itself in a departmental arrangement. What actually happens to trigger this whole change of arrangement? There is a policy decision here by the minister, who has opened this up and says, ‘It’s okay now—we’ve got countries that had BSE, but that’s okay, they can have the opportunity to apply.’

Senator HEFFERNAN—Against the background, while you are thinking about that, that Minister Crean said this was driven by the beef industry. It is here in a press release. He has quoted Lucy Knight. You blokes have said today that it was driven by the industry. Do you agree with that? Who drove it? Come on, who drove it?

Mr Yeend—I have said that, over a number of years, there have been concerns expressed by industry and—

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Senator HEFFERNAN—No—we will go back to the Hansard. You said it was triggered by the industry—words to that effect.

Mr Yeend—I did not say that. I said that the industry, over a number of years, has been expressing concerns. There have also been other factors; there have been the changes in the science—that has moved on—

Senator HEFFERNAN—Then I will ask you—

CHAIR—No, let him finish.

Mr Yeend—and also there have been concerns raised by some of our trading partners. All of these factors were considerations. But the driving factor in why the government commenced this review process was that, through the regular industry consultation that takes place, these issues had been raised, and raised, and then, in August—

Senator HEFFERNAN—In other words, it would be fair to interpret what you are saying as, ‘It was driven by the industry.’ Is that what you are telling me? I take that to be what you are saying.

Mr Yeend—I am saying that industry, through the consultation process, has raised concerns, and industry was wanting us to review the policy to see whether the current arrangements were appropriate or not.

Senator HEFFERNAN—Could you provide the advice from the industry that triggered this? Mr Yeend—I think we can detail the contact we have had with the industry. Senator HEFFERNAN—No, no—it would have had to be a written request to make such a catastrophic change of direction of the beef industry. You say it was driven by continuous pleadings from the industry and that that eventually triggered the government to do it; could you show us the continuous pleadings?

Mr Yeend—I think we could outline to you what I have been talking about.

Senator HEFFERNAN—But is there correspondence that supports what you are saying as more than hearsay?

Mr Yeend—There has been contact between the government and the industry.

Senator HEFFERNAN—No, I am saying: is there written evidence that what you are saying is not just hearsay?

CHAIR—Could the committee have any record of that contact is what we are asking.

Mr Yeend—Yes, I think we can provide you with detail of the contact there has been between government and industry through the consultation process.

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CHAIR—All right. It is not just today and not just your evidence, Mr Yeend; it has been coming through quite clearly that there has been a very strong indication that this has been driven by industry. Would you say, then, that if industry had not driven it we would not be in this position today? Or would the departments have seen this as the best course forward and given that advice to the minister anyway?

Mr Yeend—What I am saying is that the concerns expressed by industry were a key factor in the decision to change the policy, but there were a combination of factors. You are asking me a hypothetical question but, from the perspective of the issues for which I have responsibility, this issue has been raised with us by our trading partners; concerns have been expressed both by trading partners and domestically that the science has moved on and it was something that needed to be looked at again. There were questions of consistency with our international trade obligations.

Senator HEFFERNAN—You are too well-trained as a bureaucrat. That will do.

Mr Yeend—So all of—

CHAIR—That is fine, Mr Yeend. Thank you. Senator Heffernan has the call.

Senator HEFFERNAN—Stop there, mate. Stop there. You are talking gobbledygook.

According to your minister, Minister Crean: IT was the Australian beef industry’s lobbying that triggered this week’s decision to change food policy laws … So could you show us written evidence of the lobbying that Mr Crean refers to?

Mr Yeend—I think I have already said to Senator Nash that we will be happy to provide you with more information, but I would refer you to paragraph 6 of our submission that I think does say that the timing of the specific request from AHMAC was related to a discussion on 28 July 2009 within an AHMAC meeting. So I think we have addressed these issues on a number of occasions and we have put it in writing. I am happy to elaborate on it if you would like me to.

Senator HEFFERNAN—Did you say, Mr Morris, that the change of policy was announced on 20 October?

Mr Yeend—Yes, that is correct.

Senator HEFFERNAN—And yet, in earlier evidence, Professor Mathews said he did not send in his draft report until the 20th—so you made the decision before you got the draft report?

Mr Yeend—That is not correct.

CHAIR—Well, which one is not correct?

Senator HEFFERNAN—Which one is telling a lie?

CHAIR—Professor Mathews said it was 20 October—or September?

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Senator HEFFERNAN—Who is lying?

snip...

McCutcheon—If we are not satisfied with the country’s traceability requirements—

CHAIR—No, I am not even talking about countries. I am talking about individual exporters.

Mr McCutcheon—We do not look at individuals; we look at countries.

CHAIR—Who does? Who looks at the individuals?

Mr McCutcheon—That is the responsibility of the individual country making an assessment.

Senator HEFFERNAN—But if the evidence—

Senator BACK—Can I just stay with this for a second? I thought the responsibility rested with Australian authorities, not that it was the responsibility of other countries. I hope we are not divesting this to other countries to assure us that everything is in place. Are we not going to actually satisfy the Australian consumer by being able to say that Australian people under your direction are going to be satisfied? We are not going to be satisfied by some statement of an overseas country’s inspectorate, are we?

Mr McCutcheon—The first step will be for us to assess what traceability mechanisms a country has and how effectively they are claimed to be used.

Senator BACK—And if they do not have—

Mr McCutcheon—Second, if we are not satisfied then we do have the scope to go and visit countries for an inspection. I will ask AQIS to jump in here, but basically if a country is put into category 1 or 2 and they are deemed to be eligible to export to Australia, subject to all the other sanitary requirements being met, then it is another country’s responsibility to make sure that they satisfy our requirements. But, again, that is getting into AQIS territory.

Senator HEFFERNAN—Rather than mince your words, the US and Canada do not have full traceability at all. They have no traceability, as Senator Back points out, from the day an animal was born to when it was killed.

Senator BACK—Where would that place them?

Senator HEFFERNAN—So are they knocked out automatically?

Mr McCutcheon—If Canada or the US lodge an application then we will be examining traceability as part of that.

Senator HEFFERNAN—For God’s sake, we have been here for two hours talking bureaucratic nonspeak. Wouldn’t it be reasonable for Australia’s cattle producers to expect you fellows, who are in charge of this without reference to the parliament or the industry, to at least demand full traceability? Isn’t that a reasonable request?

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Senator BACK—Can I ask the question—

Senator HEFFERNAN—No, just let him answer that.

Mr McCutcheon—I think I have already answered that question. I said we will need to be satisfied that the traceability arrangements that a country have manage the risks associated with BSE.

Senator HEFFERNAN—That is bureaucratic crap. They do not have any.

snip...

snip...see full text 110 pages ;

http://www.aph.gov.au/hansard/senate/commttee/S12742.pdf

Backflip over mad cow beef ban

Nicola Berkovic

From: The Australian March 09, 2010 12:00AM

THE importation of beef from countries that have had outbreaks of mad cow disease will be delayed by at least two years following a backdown by the Rudd government. Agriculture Minister Tony Burke yesterday bowed to community pressure and ordered a full risk analysis of beef imports from countries where bovine spongiform encephalopathy, or mad cow disease, had been reported.

The review comes eight days after the lifting of a ban on beef imports from countries that have had the disease.

That decision followed a threat by meat exporters, including Canada, to go to the World Trade Organisation over unfair trade barriers.

A spokeswoman for Mr Burke said the review would cover fresh or frozen beef, but not processed or cooked beef products, such as oxtail soup, and drinks such as the beef tea product Bovril.

The review, to be conducted by Biosecurity Australia, will examine beef imports from all countries other than New Zealand.

Start of sidebar. Skip to end of sidebar. Related CoverageRisky red meat imports shelved Daily Telegraph, 3 hours ago Turnaround sees risk study on beef imports Adelaide Now, 10 hours ago Oxtail soup first beef import The Australian, 5 days ago Labor to axe drought relief The Australian, 6 days ago N American push on mad cow ban The Australian, 24 Feb 2010 .End of sidebar. Return to start of sidebar. Mr Burke said he had taken the decision following "significant community concern" about import standards.

Admitting it was unusual for a minister to intervene in such a decision, Mr Burke said the level of concern had warranted the formal review process.

"Conducting an import risk analysis is the best way of reassuring the Australian community that effective protocols will be put in place to provide for the safety of imports."

He said he believed the two-year review process would not put Australia in breach of its international trade obligations, because it was a science-based procedure.

Independent senator Nick Xenophon, who campaigned against the lifting of the ban, welcomed the review as a "victory for commonsense".

"The importation of meat from mad cow disease-affected areas could have potentially put Australian consumers at risk, and could have destroyed Australia's claims that we are a clean, green, BSE-free market," Senator Xenophon said.

Nationals senators Fiona Nash and John Williams also hailed the decision as a sensible move to protect consumers and beef producers.

However, the Cattle Council of Australia, which argued that the analysis would only burden international trade, said it was disappointed a protectionist "scare campaign" influenced the decision.

President Greg Brown said he was worried about the delay the process would create. "If the Americans put a 24-month process on us, we would be out of the market . . . considering they take a hell of a lot more beef from us than we do from them," he said.

The confusion over beef imports had also damaged the reputation of Australian beef in the domestic market, Mr Brown said.

Australia banned British beef in 1996.

http://www.theaustralian.com.au/news/nation/backflip-over-mad-cow-beef-ban/story-e6frg6nf-1225838417701

WORK WITH OTHER INTERNATIONAL ORGANIZATIONS

The WTO and the World Organization for Animal Health (OIE)

Collaboration between WTO and OIE concerns the use of international standards in the context of the SPS Agreement.

o ON THIS PAGE

Mandate The OIE STDF

See also: OIE website

Note: The World Organization for Animal Health was originally called the Office International des Epizooties, and is referred to as such in the SPS Agreement

Mandate back to top

The WTO's SPS Agreement states that “to harmonize sanitary and phytosanitary measures on as wide a basis as possible, Members shall base their sanitary or phytosanitary measures on international standards, guidelines or recommendations”. The Agreement names the OIE as the relevant organization for animal health.

A formal cooperation between OIE and WTO was agreed in July 1998 WT/L/272

See SPS Agreement Introduction, Article 12.3 and Annex A paragraph 3(a)

http://www.wto.org/english/thewto_e/coher_e/wto_oie_e.htm

The OIE and the WTO strengthen their cooperation

Paris, 14 March 2009 – During a visit by Mr Pascal Lamy, Director-General of the World Trade Organization (WTO), to the OIE Headquarters in Paris, the leaders of the two organisations further emphasised the benefits to the international community of the animal health standards published by the OIE, which are considered as the reference within the framework of the WTO SPS Agreement.

Dr Bernard Vallat, Director General of the OIE, stated that “a large majority of the standards adopted by OIE Member Countries and Territories are designed to prevent sanitary risks linked with the world trade in animals and animal products. This trade is indispensable, especially to supply the poorest countries with animal protein, including in times of crisis. Yet compliance with OIE standards also results in the improvement of veterinary health governance in each Member country and territory, and in so doing improves animal health and welfare throughout the world, while at the same time improving public health by preventing and controlling animal diseases transmissible to humans.”

The two organisations also raised the need for joint discussions on how to avoid the potential disadvantages arising from the use of “private” standards relating to sanitary risks, since such standards have not been adopted within the framework of the SPS Agreement and may contradict existing public standards democratically adopted by the OIE and the Codex Alimentarius Commission in a fully transparent procedure and based on scientific evidence.

The OIE also referred to its standard-setting work in the field of animal welfare and the rise in stricter consumer demands in this respect in all countries of the world.

In the field of improving the competencies of stakeholders in all countries of the world on international sanitary rules applicable to trade, the WTO and the OIE reaffirmed their commitment to the Doha Declaration issued by the WTO, OIE, WHO, the World Bank and FAO, and to the Standards and Trade Development Facility (STDF) to help developing countries fulfil the requirements of the SPS Agreement.

“The value of the WTO organising information and training seminars in all regions with the OIE for the benefit of Veterinary Service officials, and especially those in charge of sanitary certification of animals and animal products for export, is now a well established fact. These seminars should be continued, along with the allocation of STDF grants aimed at facilitating the preparation of national or regional projects to strengthen the sanitary safety of international trade and market access opportunities for all those that so wish”, declared Pascal Lamy.

Lastly, the two organisations reiterated their shared interest in strengthening collaboration in order to facilitate the settlement of sanitary disputes, notably by making more frequent use of the OIE mediation procedure.

March 2009

http://www.oie.int/Eng/press/en_090314.htm

THE O.I.E. IS nothing more than an industry group set up to abolish all protocols for the safety of humans from all strains of Transmissible Spongiform Encephalopathy. The O.I.E., the U.S.D.A., and evidently the W.T.O. have all come to the conclusion, that the trading of all strains of mad cow disease and or any animal Transmissible Spongiform Encephalopathy should now legal. TSEs are now nothing more than a commodity, a legally traded commodity, and any body bags there from are simply a casualty of trade and the almighty dollar. humans have now become expendable. AFTER what happened with Global trade and typical BSE, the governing bodies of global trade have pressed forward with the fact (without any scientific proof whatsoever), that any atypical TSE is nothing more than an spontaneous old animal disease, and that it has become an acceptable trading commodity, even though we know that atypical BSE is transmissible to humans (Kong et al 2009). these trading partners have manipulated science to be able to trade these TSE globally, and any ramifications there from is simply an acceptable death either from direct consumption, or the 'friendly fire' i.e. 'pass it forward' mode of transmission. i kinda compare the O.I.E., W.T.O., U.S.D.A. et al with the Mafia. no i am not kidding. have you ever ead the 'mad sheep of mad river valley' ?

Saturday, February 27, 2010

FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010

http://foiamadsheepmadrivervalley.blogspot.com/2010/02/final-report-of-testing-of-belgian.html

see history here ;

http://foiamadsheepmadrivervalley.blogspot.com/

odd how when c-BSE was linked with nvCJD of humans with pathology that is very similar to each other, they confirmed a link between humans. HOWEVER, with the atypical Nor-98 scrapie being very similar to some sub-types of sporadic CJD, and GSS, it's just the opposite, its all spontaneous $$$ will the corruption ever end ?

BOTTOM LINE ABOUT THE O.I.E.

IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............

http://www.oie.int/eng/Session2007/RF2006.pdf

IN OTHER WORDS, THE O.I.E. TAKES WHATEVER JUNK SCIENCE THAT A COUNTRY SUBMITS TO THEM, AND THEY CLASSIFY THAT COUNTRY BY THAT JUNK SCIENCE. it's like the wolf guarding the henhouse. it would be like the big bad wolf coming to the door and saying nobody is home. and you believing them, and then basing the BSE risk assessment on that. WE ALL know what kind of junk science the USDA and the FDA et al have used over the years. ...TSS

Posted: Wed Dec 19, 2007 3:47 pm Post subject: OIE

--------------------------------------------------------------------------------

Question wrote:

Quote: Maybe familirise yourself with the OIE. The primary concern is animal health of the world they are the animal version of the WHO. It is a long way down from that ivory tower but here we go, until pressured by the USA repesentatives a country could not export animals for 6 years after finding a BSE/BASE positive animal so under the old rules the US would not be able to export anywhere in the world for another 4 1/2 years. Who got the risk levels system put in to allow some trade - your US representatives. You guys want to change rules - OK , but you do not get special rules that only apply to the US. As i have told you before Sand h I market all my own slaughter animals and you know that, so don't do the whole holier than thow act.

With all due respect, it is obvious that you know little about the OIE and how it actually works. Having been to their offices in Paris and talked personally with the Head of the Animal Test Section, you would choke if you knew how many lobby groups attend that office daily. There is a steady stream of paid lobby groups that have one goal in life and that is to sway the Section Heads of each department within the OIE to suit the needs of different juristictions around the world, which curiously enough, also includes the USA and Canada. Anyone can go there and chat with them - providing they can privide valid cause to be let in. To say that the only goal of the OIE is animal health is actually only part of their function. They are more than that and my discussions with Dr. Diaz there has showed me that. But to blindly make a statement regarding what they do when you have no idea what they actually do is like eating the skin of the orange and not knowing what is actually under.

Interstingly you state that the US Government applied pressure (to the OIE) I assume and that is a great example of the lobby groups doing their job. So, at the end of the day, one can safely assume that it is the pressure applied by certain influential lobby groups that will determine a likely aoutcome to an apparent OIE directive. Man alive, isn't it great to live in a democracy wherein the people get to make the choices and not just some "other" interested party or group - say like........Cargyll or Tyson for example?

So, one last question, question?

Who wags the tail of that dog?? And for what reason other than one that is purely associated with trade and international agreements and greed?

And you think it is so simply explainable.

http://www.ranchers.net/forum/viewtopic.php?t=22833&postdays=0&postorder=asc&highlight=ops&start=36

SCRAPIE The United States is unable to support the proposed new draft Code Chapter on Scrapie. The draft chapter, as written, departs significantly from the existing chapter, is confusing and is difficult to understand. This version of the scrapie chapter uses much of the same wording as the BSE chapter and is written as if the predominance of evidence revealed that scrapie was a food-borne disease similar to BSE in cattle which is inappropriate. Moreover, several of the new changes are not supported by current scientific evidence. As a result, detailed comments on individual articles would not meaningful at this time. The United States is not supportive of the proposed draft chapter for the following reasons: 1. Inclusion of “atypical” scrapie: The scientific evidence indicates that “atypical” scrapie, also referred to as Nor-98, Nor-98-like, or non-classical scrapie, is not the same disease as classical scrapie. Further, “atypical” scrapie does not meet the criteria for listing diseases of trade concern by the OIE, as described in Chapter 2.1.1 of the Code. The United States recommends that the scope of this chapter be limited to classical scrapie in sheep and goats. Further, the United States recommends that OIE clearly adopt the position that “atypical” scrapie represents a distinct disease entity from classical scrapie and that it not be a listed disease. • There is no evidence that “atypical” scrapie is a contagious disease. If it is contagious, available evidence suggests that it has a much lower transmission efficiency. (Hopp, et al, 2006; Green, et al, 2007; Benestad, et al 2008; McIntyre, et al, 2008) • The disease appears to be ubiquitous in that it has been found wherever sufficient surveillance has been conducted. (Buschmann et al, 2004; De Bosschere et al, 2004; Orge, et al, 2004; Everest et al, 2006; Arsac, 2007; Benestad, et al 2008; Fediaevsky, et al, 2008) • The disease does not appear to be economically significant in that the prevalence of clinical disease is low and it typically occurs in older animals. (Luhken, et al., 2007; Benestad, et al 2008). • The disease is as likely as not to be the result of a spontaneous conversion of normal prion protein. (Benestad, et al 2008, De Bosschere et al 2007) • Removal of exposed sheep is unlikely to reduce the prevalence of “atypical” scrapie infection and removing only those exposed sheep that are phenylalanine (F) at codon 141 is scientifically unsound since the disease is known to affect sheep of most other genotypes. Further, sheep with AHQ alleles have a similar risk of infection with “atypical” strains as sheep with F at codon 141. (Luhken, et al., 2007). • If “atypical” scrapie is included as a listed disease, the surveillance and diagnostic requirements which are needed to identify these cases should be described in detail in both this Chapter and the Manual of Diagnostic Tests and Vaccines for Terrestrial 2 Animals. Data from Europe illustrates that using the proper test(s) is essential for the identification of atypical scrapie (Fediaevsky et al., 2008).

SNIP...

6. Overemphasis on importation and use of bovine meat and bone meal as a route of scrapie transmission: Given that the draft Chapter is not intended to address risk mitigation for BSE in small ruminants, we believe there is an over-emphasis on this potential route of transmission in the current draft. The United States recommends that the requirements in this chapter be limited to the inclusion of products from sheep and goats (instead of from all ruminants) in feed or feed ingredients intended for consumption by animals • The use of products from sheep and goats as feed or feed ingredients for ruminant or non-ruminant animals represent one possible route of transmission (Philippe, et al, 2005) and a source of environmental contamination with the classical scrapie agent. However, this is not the primary route of transmission for the scrapie agent. • The need for the exclusion of cattle-derived protein or other animal protein to mitigate BSE risk should be based on a country’s BSE risk status and should be addressed in Chapter 2.3.13 of the Code.

SNIP...

14. Failure to provide scientific justification for the list of permitted commodities in Item 1 of Article 2.4.8.1. . We recommend that the list be re-evaluated and those items that have not been substantiated as presenting no risk be excluded or those with some risk but where the intended use mitigates the risk the use be specified. • There is no known human health risk associated with scrapie. As such, if meat and meat products for human consumption are included in this list, sheep and/or goat milk intended for human consumption should also be added to the list of permitted commodities in Item 1 of Article 2.4.8.1. • In the vast majority of sheep infected with classical scrapie, actual infectivity or PrPres has been identified in most tissues including the lymphoreticular system (tonsils, spleen, lymph nodes), the gastrointestinal tract, brain, and spinal cord (Hadlow et. al. 1979; Hadlow et al., 1980; van Kuelen et al., 1996; van Kuelen et al., 1999, Andreoletti et al., 2000; Heggebø et al., 2002; Caplazi et al., 2004). Infectivity and/or PrPres has also been identified in the placenta (see Hourrigan et al., 1979; Onodera et al., 1993; Pattison et al., 1972; Pattison et al., 1974; Race et al., 1998), blood (Hunter et al., 2002; Houston et al. 2008); peripheral nerves (Groschup et al., 1996), muscle (Pattison and Millson, 1962; Andreoletti et al., 2004; Casalone et al., 2005), salivary gland (Hadlow et al., 1980; Vascellari et al., 2007), kidney (Siso et al., 2006), and skin ( Thomzig et al., 2007). In addition, recent work has shown milk and/or colostrum from scrapie infected ewes transmitted the disease to 17 of 18 lambs (Konold et al., 2008). • The data on the risk of low protein tallow made from scrapie infected tissues particularly for use in milk replacer is limited and some epidemiologic studies suggest an association of milk replacer use with scrapie risk. Taylor et al., 1997 examined the inactivation capacity of different rendering system in regards to scrapie. The presence of infectivity was determined by bioassay into mice. From the onset of this study, it was assumed that tallow was not the vehicle for the transmission of TSE. Hence only 2 tallow samples were examined.

http://www.aphis.usda.gov/import_export/animals/oie/downloads/tahc_mar-sep08/tahc-scrapie-77-mar08_cmt.pdf

REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION Paris, 10-14 March 2008

The OIE Terrestrial Animal Health Standards Commission (the Code Commission) met at the OIE Headquarters in Paris from 10 to 14 March 2008.

SNIP...

13. Bovine spongiform encephalopathy (BSE) a) BSE (Chapter 2.3.13.) EN EN The Code Commission received comments from Argentina, Australia, Canada, the EU, Japan, New Zealand, the People’s Republic of China and the USA, and industry organisations. The Code Commission again expressed its concern that Members continued to resubmit comments on texts already discussed and adopted in previous meetings without providing any new justification. With regard to the request of Members to modify the text currently found in Article 1, the Code Commission noted that the European Food Safety Authority (EFSA) has conducted a study on the production of protein free tallow. The results of this study have already been considered by the Code Commission and found not to provide justification for modifying the current text. Some Members raised questions about the safety of deboned muscle meat and proposed that this be removed from Article 1, while other Members are questioning the limitation to 30 months of age. The Code Commission reminded Members that the measures relating to the safety of deboned muscle meat were formulated several years ago, when the magnitude of risk to human health was unknown. These precautionary measures were appropriate at that time. Since that time, scientific understanding regarding the BSE risk classification of countries and the risk to human health associated with BSE in bovine products has progressed. The Code Commission considered that it is timely to reconsider whether there is any need to maintain the current requirement in Chapter 2.3.13. for cattle to be 30 months of age or less for deboned muscle meat to be considered a safe commodity. The Code Commission agreed with Member comments regarding the need to provide annual updates to support the retention of countries/zones on the list of negligible or controlled risk countries and zones and modified Articles 3 and 4 accordingly. Members again raised comments on Article 7, proposing to modify this Article by adding the following text: ‘or after the date of birth of the last indigenous case if that indigenous case was born after the date of the feed ban’. The Code Commission disagreed with this proposed modification as this principle is already covered in Article 7, i.e. the birth of an indigenous case is an indication that the feed ban has not been effective and the relevant date would be adjusted accordingly. On the safety of gelatine, the Code Commission reiterated its position that the safety of the gelatine manufacturing process has been well established by peer-reviewed scientific studies and risk assessments on the production of gelatine from bones, regardless of their origin. Recognizing the fact that skulls are not used in the commercial manufacture of gelatine, the Code Commission proposed the exclusion of skulls, thus removing the point of contention raised by Members. Relevant references include the following: Grobben AH, Steele PJ, Somerville RA, Taylor DM (2004). Inactivation of the bovine spongiform encephalopathy (BSE) agent by the acid and alkaline processes used in the manufacture of bone gelatin. Biotechnology and Applied Biochemistry, 39; 329-338. EN EN Grobben AH, Steel PJ, Taylor DM, Somerville RA, Schreuder BEC (2005). Inactivation of the BSE agent by heat and pressure process for manufacturing gelatin. Veterinary Record, 157; 277-289. Grobben AH, Steele PH, Somerville RA, Taylor DM (2006). Inactivation of transmissible spongiform encephalopathy agents during the manufacture of dicalcium phosphate from bone. Veterinary Record, 158; 361- 366. NZFSA (2005). Officials’ Review of New Zealand’s BSE Country-Categorisation Measure. New Zealand Food Safety Authority, Wellington and published in “Prions in Humans and Animals”. Ed. Hornlimann, B., Riesner, D. Kretzschmar, H. De Gruyter Verlag, Berlin (ISBN 978-3-11-018275-0) The Code Commission noted that the approach taken to gelatine in Chapter 2.3.13. is fully consistent with approaches elsewhere in the Code whereby commodities originating from countries/zones that are not free of specified diseases are identified as safe for trade, based on the processing of the commodity as evaluated by scientific studies and risk assessment. b) Guidelines on surveillance for BSE (Appendix 3.8.4.) The Code Commission received comments from Argentina, Canada, the EU, New Zealand and the People’s Republic of China. The Code Commission accepted a comment on Article 4, a modification to correct a typographical error in Table 2 (Surveillance Point Values for Samples Collected from Animals in the Given Subpopulation and Age Category). c) Factors to consider in conducting the BSE risk analysis in Chapter 2.3.13. (Appendix 3.8.5.) The Code Commission received comments from Argentina. The Code Commission also considered recommendations of the ad hoc Group on Atypical Scrapie and Atypical Bovine Spongiform Encephalopathy, as endorsed by the Scientific Commission, and modified texts accordingly. The revised texts are presented at Annex XV of this report for adoption. Community position: The Community would like to stress in particular the comments related to the ruminant to ruminant feed ban provisions (Article 2.3.13.3, 4, 7, 8, 9 and 10), SRM definition (Article 2.3.13.14, 15, 16, 16bis), tallow (Article 2.3.13.1.e), the annual update (Article 2.3.13.3.), gelatine (Article 2.3.13.15) and the use of the risk assessment guidelines (Article 3.8.5.1. of Appendix 3.8.5.). Thus the Community cannot support the proposed chapter, and wishes its comments to be taken into account.

snip...

30. Scrapie (Chapter 2.4.8) The Code Commission thanked the Scientific Department for convening relevant experts and providing advice on atypical scrapie and atypical bovine spongiform encephalopathy. The Code Commission noted the statement in the report of the ad hoc Group to the effect that there is insufficient information to support the establishment of rules or guidelines specific to atypical scrapie, other than in relation to the choice of diagnostic tests used for surveillance. The ad hoc Group also observed that scrapie does not represent a public health risk and should not therefore be treated in the same way as BSE. The Code Commission noted that the revised draft Chapter represents a helpful step towards updating the Code’s provisions on scrapie, bringing it into line with the structure of the BSE Chapter. To facilitate review, the texts are presented as clean texts. The revised text is presented at Annex XXXIII for Member comments. Community position: The Community will provide the OIE with comments before the 15th of August 2008.

http://ec.europa.eu/food/international/organisations/eu_written_comments_terrestrial_code_may08.pdf

OIE Scrapie Chapter Revision • Current draft recognizes Nor98-like scrapie as a separate disease from classical scrapie • USDA provided comments on the draft to OIE

http://www.animalagriculture.org/Solutions/Proceedings/Annual%20Meeting/2009/Sheep%20&%20Goat/Myers,%20Thomas.pdf

Atypical scrapie/Nor 98 October 2009

Last year, after examining member country submissions and investigating rigorous scientific research, the World Organisation for Animal Health (OIE) decided that Nor 98 should not be listed in its Terrestrial Animal Health Code. The Code sets out trade recommendations or restrictions for listed diseases or conditions, and the OIE determined there was no need for such recommendations around Nor 98.

http://www.nzfsa.govt.nz/publications/ce-column/ce-web-nor98.htm

http://www.biosecurity.govt.nz/files/pests/atypical-scrapie/atypical-scrapie-faq-oct09.pdf

Sutton reported that USDA has urged the World Organization for Animal Health (OIE) to categorize Nor98-like scrapie as a separate disease from classical scrapie. Currently, the OIE has proposed a draft revision of their scrapie chapter that would exclude Nor98-like scrapie from the chapter. USDA will be submitting it's comments on this proposal soon.

http://www.ohiosheep.org/Events/ScrapieNewsletterMarch09.pdf

Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html

EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE

This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........

http://web.archive.org/web/20010305222246/www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.

The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.

It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html

P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf

PR-26

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.

119

http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf

A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

http://www.pnas.org/content/102/44/16031.abstract

Monday, December 1, 2008

When Atypical Scrapie cross species barriers

Authors

Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.

Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.

(i) the unsuspected potential abilities of atypical scrapie to cross species barriers

(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier

These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

“The U.S. has lower sanitary and phyto-sanitary standards (SPS) for imports than many other countries, especially those concerning bovine spongiform encephalopathy (BSE). These low standards have made the U.S. a dumping ground for beef from the countries that have experienced BSE problems. Food Safety and SPS issues continue to be problematic for our industry, as some countries comply with OIE standards, while others ignore them either for cultural reasons, or too often use them as trade barriers. The USITC October 7, 2008 release reported, ‘U.S. beef processors and beef cattle ranchers lose billions of dollars in export opportunities each year because of animal health and food safety measures in other countries that are inconsistent with international standards and vary by country.

http://www.cattlenetwork.com/USCA-Testifies--Before-USITC/2010-03-03/Article_Latest_News.aspx?oid=996238&fid=CN-LATEST_NEWS_

Epidemiology of Scrapie in the United States 1977

http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf

Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010

http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html

Thursday, March 11, 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010

http://nor-98.blogspot.com/2010/03/canada-typical-and-atypical-scrapie.html

Tuesday, April 28, 2009

Nor98-like Scrapie in the United States of America

http://nor-98.blogspot.com/2009/04/nor98-like-scrapie-in-united-states-of.html

Scrapie USA

http://scrapie-usa.blogspot.com/

LIKE i said before, the OIE not only sold their soul to the devil over the BSE MRR, they sold yours too ;

Wednesday, February 10, 2010

NAIS MAD COW TRACEABILITY DUMPED BY USDA APHIS 2010

http://naiscoolyes.blogspot.com/2010/02/nais-mad-cow-traceability-dumped-by.html

The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of the USA Question number: EFSA-Q-2003-083

Adopted: 1 July 2004 Summary (0.1Mb)

Report (0.2Mb)

Summary

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.

A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1211902594180.htm

Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html

Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801e47e1

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028 Public Submission Title Comment from Terry S Singletary

Comment 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.

MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure. ...

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8151

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8

THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.

...snip

IT'S as obvious as day and night, either Larry, Curley, and Mo have been at the helm of the USDA/APHIS/FSIS/FDA/CDC/NIH et al for many many years, or the incompetence of these agencies are so inept, either through ignorance and or just too overweight with industry reps., they then should be all done away with and a single agency brought forth, and if not, how will you correct this ongoing problem ?

Thank you, I am sincerely disgusted,

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

...snip

full text ;

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html

PLEASE SEE FULL TEXT 98 PAGES HERE ;

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN

''they don't wanna know, the dont' care''

''nothing else matters''

watch and listen to Stanley Prusiner, turn it up ;

http://maddeer.org/video/embedded/prusinerclip.html

Prions: Protein Aggregation and Infectious Diseases ADRIANO AGUZZI AND ANNA MARIA CALELLA Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland

3. Sporadic Creutzfeldt-Jakob disease Approximately 85% of all human prion diseases are sporadic forms of CJD. For sCJD, there is no association with a mutant PRNP allele, nor is there any epidemiological evidence for exposure to a TSE agent through contact with people or animals infected with TSEs. sCJD cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the size and glycoform ratio of proteaseresistant prion protein identified on western blot (type 1 or type 2) (174). Heterozygosity (Met/Val) at PrP codon 129 appears to be associated with a lower risk (378) and/or prolonged incubation time (119, 387). The lack of routine laboratory testing for preclinical diagnosis makes the search for agent sources and other risk factors extremely difficult. At present, the means of acquisition of a TSE agent in these patients remains a mystery. So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE. In humans, the peak age incidence of sporadic CJD is 55–60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

Physiol Rev • VOL 89 • OCTOBER 2009 • www.prv.org

Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro*

Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either "prion diseases" because of the association of a misfolded cellular prion protein in pathogenesis or "transmissible spongiform encephalopathies" (TSEs) because of the spongelike nature of the damaged brain tissue (1).

The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by "spontaneous" misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or "prion."

Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). BSE caused by spontaneous misfolding of the prion protein has not been proven. CREDIT: KATHARINE SUTLIFF/SCIENCE

snip...

Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent--unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD--which is considered by some researchers to begin by spontaneous misfolding of the prion protein--usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady. ...

snip...full text ;

http://www.sciencemag.org/cgi/content/full/sci;305/5692/1918

Release No. 0106.04

Contact: Office of Communications (202) 720-4623

Transcript of Remarks From Technical Briefing on BSE and Related Issues With Agriculture Secretary Ann M. Veneman and USDA Chief Veterinary Officer Dr. Ron DeHaven Washington D.C. - March 15, 2004

snip...

OPERATOR : “Yes. Our next one is coming from Elizabeth Weiss. Please state your company.”

ELIZABETH WEISS: “This is Elizabeth Weiss with USA Today.”

“I actually had two questions. First off, when you say you're looking for 1 in 10,000 cases, is USDA doing any work to find out the possibility of whether or not BSE exists in a spontaneous form in the way that it does in humans and elk populations?

“And secondly, how will any of this fit into some of the consternation that's been raised in California and with the Midwest packer that wanted to test all of its cattle?

“Thanks.”

DR. DEHAVEN: “All right. I think we've got three different questions in there, and I'll try to touch on each one of them.

“First of all, let me correct just a technical issue, and that is you mentioned 1 in 10,000. And actually our surveillance system currently is designed, the one that we have in place now is designed to detect 1 positive in 1 million cattle, and I gave some numbers between 200,000 and 268,000 that would allow us to detect 1 in 10 million as opposed to 1 in 10,000.

“So we would, if we were able to collect in the ballpark of those numbers of samples then we with increasing numbers of samples have an increasingly statistically valid sample from which to determine, one, whether or not the disease exists and, if so, at what prevalence level.

“So our real emphasis is to test as many of those animals as we can, ensure that we get an appropriate geographical distribution, but not setting a specific number as far as a target. Again, consistent with the recommendation from the International Review Team, their recommendation was to test all of them.

“So that's consistent with where we're going is to test as many as we possibly can.

“As far as spontaneous cases, that is a very difficult issue. There is no evidence to prove that spontaneous BSE occurs in cattle; but here again it's an issue of proving a negative. We do know that CJD, the human version of the disease, does occur spontaneously in humans at the rate of about 1 in 1 million. We don't have enough data to definitively say that spontaneous cases of BSE in cattle occur or do not occur.

“Again, it's a very difficult situation to prove a negative.

“So a lot of research is ongoing. Certainly if we do come up with any positive samples in the course of this surveillance we will be looking at that question in evaluating those samples but no scientifically hard evidence to confirm or refute whether or not spontaneous cases of BSE occur.

snip...

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2004/03/0106.html

UK TESTING FINISHED NEXT WEEK

Matthews confirmed that the brain tissue samples from the US animal had arrived at Weybridge. Test results were likely to be ready by the end of next week, he said.

The suspect animal has already undergone a series of tests. A rapid screening test on Nov. 15 returned inconclusive results. Sophisticated immunohistochemistry (IHC) tests cleared the animal of any infection, but a third round of testing using a Western blot procedure showed a "weak positive".

Weybridge will do an IHC test plus three kinds of Western Blot tests on the samples. They will use "methods of slightly different analytical sensitivity that give us the greatest number of opportunities to interpret what we see," he said.

US beef industry leaders say scientists should not speculate about the unusual case.

"There's no evidence that it's atypical ... and there's absolutely no evidence that it's spontaneous," said Gary Weber, head of regulatory affairs at the National Cattlemen's Beef Association.

Matthews noted scientists are still grappling with what is typical and atypical BSE.

"Far too few people have looked at BSE in depth using all of the tests to be able to define 'this is normal and that one isn't'," he said.

Weber noted Japan used the term to describe two very young infected cattle because BSE is usually found in older animals. Italy labeled a case "atypical" because the misshaped prions were found in unexpected parts of the animal's brain. ...snip...end

http://www.agobservatory.org/headlines.cfm?refID=73207

Atypical BSE strain -- In July 2007, the UK Spongiform Encephalopathy Advisory Committee (SEAC) suggested that atypical BSE may be a distinct strain of prion disease. Unlike typical BSE, cases of atypical BSE, according to SEAC, may have risen spontaneously (although transmission through feed or the environment cannot be ruled out). Recently reported French surveillance data support this theory that unlike typical BSE, atypical BSE appears to represent sporadic disease

http://cdc.gov/ncidod/dvrd/bse/

http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf

Spontaneous occurrence

14. Spontaneous cases of classical CJD in humans are found at a rate of about 1/million around the world (Will, 1993), without appreciable racial or geographical variation except in a few specific cases, notably Jews of Libyan origin that have a mutation in the open reading frame of the PRNP gene (Chapman and Korczyn, 1991). Thus it is theoretically possible that spontaneous cases of BSE could occur as a consequence of a germ line mutation, in which case relatives would also have a certain or increased incidence of the disease, or a somatic mutation, which would be unlikely to be detectable unless the appropriate tissue were identified, or after some transformation in the PrP protein in the animal concerned. BSE was unknown prior to its detection in Britain in the mid 1980s, and Index cases found around the world since then can all be explained in terms of export from the UK directly or indirectly of cattle or of feed components. No BSE affected animals have been reported in many developed countries with large cattle populations, including Australia, New Zealand, Norway and Sweden, which have mixed cattle populations; and the only infected animal detected in the US was of Canadian origin. The disease seems to have a highly homogeneous aetiology (e.g. Bruce, 2003). 15. Data from the USA, where the dairy population in particular is highly related to that in GB provide an upper limit to the spontaneous rate. A programme of testing is in place of a target population of adult cattle exhibiting some clinical sign that might be consistent with BSE (animals reported as having CNS or clinical signs of BSE or were non-ambulatory). In the intensive programme from June 2004 over 375000 animals were tested in the following 12 months. No positive results have yet been obtained in these or previous tests (USDA BSE Testing). This implies a putative upper limit of under 10 per million in this target group. Assuming, as analysis has shown, the relative risk in this group is about 30 times higher than in the population as a whole (European Commission, 2002c), then the incidence in the population as a whole is under 3 per 10 million. This figure could possibly be biased downwards if affected animals are diagnosed and disposed of without being tested. Taking account of testing done and the lack of clinical cases seen in many other countries also, it seems highly unlikely that the spontaneous rate can be as much as 3 per 10 million head. Nor can spontaneous occurrence explain incidences of ca. 30 cases of BARBs per year in 2002/4 in the UK adult cattle population of ca. 4 million. [NOTE ADDED 30 JUNE: The recent confirmation of a previously inconclusive case in the USA affects these calculations. If the animal did not have access to infected feed, the calculations have to be revised: they suggest a sporadic incidence in the population of 1/(375000 x 30) or almost 1 per 10 million, with the upper limit under 5 per 10 million.] 16. Calculation of a maximum rate of possible transformation from scrapie to BSE is less feasible. Nevertheless, BSE appears not to have arisen in the UK until around the early 1980s, despite the presence of scrapie in sheep here for at least 200 years. Although a change in the scrapie prion may have been the cause of the initial cases of BSE, the difference between their properties in mice and the uniformity of the BSE brain lesions suggest it is unlikely that more than one such mutation was the source of BSE. It is most unlikely that the same mutation could be occurring often enough to contribute significantly to BARBs cases. Furthermore BARBs cases do not match the geographical distribution of the sheep population. The evidence from the absence of BSE in many countries and the surveillance schemes abroad indicates that most BARBs cases cannot have arisen spontaneously, although the possibility cannot be excluded that a very

http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf

242 Atypical and classical BSE are different strains based upon Western blot profiles 243 (Hill, 2004; Normile, 2004; Baron et al., 2006), and this study indicates that disease Page 11 of 23 Journal of Animal Science Downloaded from jas.fass.org by on November 12, 2008. 12 progresses via different routes for these strains. The disparate 244 routes of pathogenesis in 245 atypical BSE can occur by 1 of 2 means. One possibility is that the source of infectivity 246 in atypical BSE is exposure to contaminated feedstuffs, as is the case for classical BSE, 247 but progression occurs in a disparate manner that bypasses the influence of the indel 248 polymorphisms. The other possibility is that atypical BSE is occurring spontaneously in 249 the host. Support for atypical BSE occurring spontaneously are the parallels to sporadic 250 TSE in humans, specifically, occurrence in older hosts and a comparable low incidence 251 rate (Baron and Biacabe, 2006). Furthermore, atypical BSE occurs as isolated, sporadic 252 cases in contrast to the clustering of cases observed for feed borne classical BSE 253 (Donnelly et al., 1997). Interestingly, the only native born cases of BSE in the United 254 States identified to date have been classified as atypical BSE.

255 No experiment can conclusively confirm a spontaneous nature for atypical BSE.

snip...end

http://jas.fass.org/cgi/reprint/jas.2007-0208v1.pdf

Monday, February 11, 2008 7:00 am Registration and Morning Coffee

Emerging Concerns: De novo Formation of Prions

9:05 De novo Generation of Prion Infectivity in a Cell-Free System Joaquin Castilla, Ph.D., Assistant Professor, Department of Infectology, Scripps Research Institute-Florida Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative disorders affecting both humans and animals. There is no available treatment or therapy for these fatal diseases. The infectious agent associated with TSEs (termed prion) appears to be composed uniquely of a protein, which is a conformationally-modified version (PrPSc) of the cellular prion protein (PrPC). The disease is propagated by the conversion of host PrPC into PrPSc induced by small quantities of PrPSc. Interestingly, prions occur in the form of different strains that show distinct biological and physicochemical properties. TSEs can have diverse origins, including genetic, sporadic (putatively spontaneous) and infectious. The occurrence of sporadic cases of prion diseases in humans and maybe in other species, i.e. atypical bovine spongiform encephalopathy (BSE) in European and USA cattle and atypical scrapie cases in sheep suggest that spontaneous prion diseases may happen infrequently but ubiquitously. However, there are no reported cases of spontaneously-occurring prion disease in experimental wild-type rodent models. We have used a novel technique, Protein Misfolding Cyclic Amplification (PMCA) to rapidly propagate prions in the test tube, using normal brain homogenate as substrate. Prions propagated in vitro are infectious in vivo and maintain their prion strain specificity. PMCA has been used to efficiently amplify a variety of prion strains from mouse, hamster, bank vole, deer, cattle, sheep and human. Therefore, to mimic spontaneous generation of infectivity in vitro becomes one of the most important challenges in the prion field. We show here, for the first time, the de novo generation of infectious prions from bank voles (Clethrionomys glareolus) starting with non-infectious brain homogenates. Several biochemically different prion strains were generated using two different wild-type vole genotypes. The de novo in vitro generated PrPSc was highly infectious after its inoculation in bank voles. We show an extensive characterization of this "spontaneous" phenomenon.

http://www.healthtech.com/Conferences_Overview.aspx?c=518&id=59662&c=518

Pathogenesis Chairperson: Suzette Priola, Ph.D.

11:10 Accumulation of Prion Protein in the Brain That is Not Associated with Transmissible Disease Pedro Piccardo, M.D., Senior Investigator, OBRR / DETTD / LBPUA, FDA (Invited)

11:35 High Levels of TSE Infectivity Can Be Associated with Little or No Detectable PrPSc in Vivo Rona Barron, Ph.D., Neuropathogenesis Unit, Roslin Institute and Royal (Dick) School of Veterinary Studies This work examines the relationship between TSE infectivity and the abnormal prion protein, PrPSc. In a mouse model of disease we have shown high titres of TSE infectivity in brain tissue which contains little or no PrP-res. We also found no evidence of other abnormal PrP isofoms such as PK-sen PrPSc. These data question the true relationship between PrPSc and TSE infectivity, and the current reliance on PrPSc as the sole diagnostic marker for TSE disease.

12:00 Conversion of the BASE Prion into the BSE Prion: The Origin of BSE? Fabrizio Tagliavini, Ph.D., Director, Division of Neurology 5 & Neuropathology, Neurological Institute "Carlo Besta" Twenty years after the identification of bovine spongiform encephalopathy (BSE), the origin of the causal agent is still unknown. This issue is of fundamental importance, since knowledge of the origin of the BSE agent is essential for prevention of future outbreak of the disease or variants thereof in cattle and other mammals. We carried out transmission studies with transgenic mice expressing bovine PrP and four lines of non-transgenic mice and found that an atypical form of spongiform encephalopathy of cattle, termed BASE or BSE-L, is caused by a prion strain distinct from that of classical BSE. Noteworthy, this newly characterized prion strain has the ability to convert into the classical BSE strain upon serial transmission to inbred mouse lines. According to these results, BASE--which is regarded as a sporadic form of prion disease in cattle--may be the origin of BSE, following conversion of the causal agent in an intermediate host.

12:45 Luncheon Technology Workshop (Sponsorship Available) or Lunch on Your Own

2:00 Sporadic CJD and Atypical BSE: Two Children of One Protein Maurizio Pocchiari, Ph.D., Director of Research, Virology, Istituto Superiore Di Sanita The identification of forms of TSE diseases in cattle caused by prion strains different from BSE has raised new concerns on the possibility that these novel agents might induce disease in humans with a phenotype resembling sporadic CJD. The analysis of the distribution of the different molecular subtypes of sporadic CJD might give some answers.

http://www.healthtech.com/Conferences_Overview.aspx?c=518&id=59662&c=518

Originally published as JGV in Press, 10.1099/vir.0.010801-0 on July 22, 2009 Originally published as JGV in Press, 10.1099/vir.0.010801-0 on June 17, 2009 J Gen Virol 90 (2009), 2563-2568; DOI 10.1099/vir.0.010801-0

Short Communication

PrPTSE in muscle-associated lymphatic tissue during the preclinical stage of mice infected orally with bovine spongiform encephalopathy

Franco Cardone1,, Achim Thomzig2,, Walter Schulz-Schaeffer3, Angelina Valanzano1, Marco Sbriccoli1, Hanin Abdel-Haq1, Silvia Graziano1, Sandra Pritzkow2, Maria Puopolo1, Paul Brown4, Michael Beekes2 and Maurizio Pocchiari1

1 Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy 2 Robert Koch-Institut (P24 – Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany 3 Prion and Dementia Research Unit, Department of Neuropathology, University Medical Center, Georg-August University Goettingen, Robert-Koch-Str. 40, 37075 Goettingen, Germany 4 7815 Exeter Road, Bethesda, MD 20814, USA

Correspondence Franco Cardone franco.cardone@iss.it

The involvement of muscles in the pathogenesis of transmissible spongiform encephalopathies (TSEs) is irregular and unpredictable. We show that the TSE-specific protein (PrPTSE) is present in muscles of mice fed with a mouse-adapted strain of bovine spongiform encephalopathy as early as 100 days post-infection, corresponding to about one-third of the incubation period. The proportion of mice with PrPTSE-positive muscles and the number of muscles involved increased as infection progressed, but never attained more than a limited distribution, even at the clinical stage of disease. The appearance of PrPTSE in muscles during the preclinical stage of disease was probably due to the haematogenous/lymphatic spread of infectivity from the gastrointestinal tract to lymphatic tissues associated with muscles, whereas in symptomatic animals, the presence of PrPTSE in the nervous system, in neuromuscular junctions and in muscle fibres suggests a centrifugal spread from the central nervous system, as already observed in other TSE models.

These authors contributed equally to this work.

http://vir.sgmjournals.org/cgi/content/abstract/90/10/2563

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

International Society for Infectious Diseases Web: http://www.isid.org

Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html

my comments to PLosone here ;

http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd

Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html

Wednesday, February 24, 2010

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th

ICID International Scientific Exchange Brochure -

http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html

http://transmissiblespongiformencephalopathy.blogspot.com/

Rangen Inc 2/11/10

Department of Health and Human Services Public Health Service Food and Drug Administration Seattle District Pacific Region 22201 23rd Drive SE Bothell, WA 98021-4421 Telephone: 425-486-8788 FAX: 425-483-4996

February 11, 2010

CERTIFIED MAIL

RETURN RECEIPT REQUESTED

In reply refer to Warning Letter SEA 10-11

Christopher T. Rangen, President Rangen, Inc. 115-13th Avenue South PO Box 706 Buhl, Idaho 83316

WARNING LETTER

Dear Mr. Rangen: On June 9-11, 2009, U.S. Food and Drug Administration (FDA) investigators inspected your animal feed manufacturing facilities located at 115-13th Avenue South, Buhl, Idaho. The inspection revealed significant deviations from the requirements set forth in Title 21, Code of Federal Regulations, Section 589.2000 (21 C.F.R. 589.2000), Animal Proteins Prohibited in Ruminant Feed. This regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE). You failed to follow the requirements of this regulation, resulting in products being manufactured and distributed by your facility that were adulterated within the meaning of section 402(a)(4) of the Federal Food, Drug, and Cosmetic Act (the Act), 21 U.S.C. § 342(a)(4), and misbranded within the meaning of section 403(a)(1) of the Act, 21 U.S.C. § 343(a)(1). Our investigation determined that adulteration resulted from the failure of your firm to provide for measures to avoid commingling or cross-contamination. The adulterated feed was subsequently misbranded because it was not properly labeled. Specifically, we found:

1. Your firm failed to provide for and use cleanout procedures or other means adequate to prevent carry-over of products that contain or may contain proteins derived from mammalian tissues into animal feed that may be used for ruminants, as required by 21 CFR 589.2000(e)(1)(iii)(B). Since your feed is prepared, packed, or held under these conditions it is, therefore, adulterated under section 402(a)(4) of the Act, 21 U.S.C. § 342(a)(4).

. Mink feed that was not labeled "Do not feed to cattle or other ruminants," in accordance with 21 CFR 589.2000(e)(1)(i) and that, therefore, might be fed to ruminants, was produced using the same equipment as aquaculture feed that contains proteins derived from mammalian tissues, such as meat and bone meal. You conducted no clean-outs or flushes of equipment to remove proteins derived from mammalian tissues that may have been present before manufacturing the mink feed that might be fed to ruminants.

. The auger trucks you used to deliver bulk mink feed which contained or may have contained proteins derived from mammalian tissues were not subject to an effective clean-out prior to their use to deliver bulk animal feed, including ruminant feed, that did not contain such materials. There were no procedures to clean the trucks to remove proteins derived from mammalian tissues before shipment of animal feeds that did not contain such materials.

2. You failed to label all products which contained or may have contained proteins derived from mammalian tissues with the statement, "Do not feed to cattle or other ruminants," as required by 21 C.F.R. 589.2000(e)(1)(i). Such products are misbranded under Section 403(a)(1) of the Act, 21 U.S.C. § 343(a)(1). The misbranded product includes bulk mink feed.

. On June 9, 2009, the investigators observed approximately (b)(4) pallets of (b)(4) 50 pound bags of (b)(4) MINK FEED, lot 06/05/09. All bagged mink feed, as well as approximately (b)(4)% of bulk mink feed, manufactured at your facility, was produced using the aquaculture feed production equipment used to produce feed containing proteins derived from mammalian tissues. Because mink feed produced using this equipment may have contained mammalian tissues, it was not properly labeled, as required by 21 C.F.R. 589.2000(e)(1)(i).

This letter is not intended to serve as an all-inclusive list of violations at your facility. As a manufacturer of materials intended for animal feed use, you are responsible for ensuring your overall operation and the products you manufacture and distribute are in compliance with the law. You should take prompt action to correct the above violations and you should establish a system whereby violations do not occur. Failure to promptly correct these violations may result in regulatory action, such seizure and/or injunction, without further notice.

We acknowledge your July 31, 2009 letter detailing procedures you had implemented or planned to implement to prevent future violations of FDA regulations relating to mammalian proteins in animal feed. In particular the letter stated that Rangen would no longer purchase meat and bone meal for use in any of its animal feeds and that existing inventories of mammalian protein ingredients would be exhausted by December 31, 2009. Division Manager, Joy Kinyon made similar assertions in the course of FDA's June 2009 inspection. The July 31, 2009 letter further set out procedures Rangen would use to remedy observed violations of FDA regulations while mammalian proteins were still being used at Rangen. Finally you explained steps taken to recover or relabel feed that may have been contaminated due to commingling resulting from your manufacturing and distribution procedures. Within fifteen (15) working days of receiving this letter you should, in writing, confirm the steps you took prior to receiving this letter and notify FDA of steps you have taken since receiving this letter to bring your firm into compliance with the law. Your response should include each step that has been taken or will be taken to correct the violations and prevent their recurrence. If corrective action cannot be completed within fifteen (15) working days, state the reason for the delay and the time frame within which the corrections will be completed. Please include copies of any available documentation demonstrating that corrections have been made.

Your written reply should be directed to Scott A. Nabe, Compliance Officer, U.S. Food and Drug Administration, 22201 23rd Drive SE, Bothell, Washington 98021-4421. If you have any questions about this letter, please contact Mr. Nabe at (425) 483-4753.

Sincerely,

/s/

Charles M. Breen District Director Seattle District

cc: Joy A. Kinyon, Division Manager, Aquaculture Feeds-General Feeds Rangen, Inc. PO Box 706 115-13th Avenue South Buhl, Idaho 83316

http://www.fda.gov/ICECI/EnforcementActions/WarningLetters/ucm201893.htm

http://madcowfeed.blogspot.com/2010/03/update-429128-lbs-feed-for-ruminant.html

Monday, March 1, 2010

ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010

http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html

Tuesday, March 2, 2010

Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA

http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html

Monday, May 4, 2009

Back to the Past With New TSE Testing Agricultural Research/May-June 2009

http://madcowtesting.blogspot.com/2009/05/back-to-past-with-new-tse-testing.html

I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''

Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS

Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620

http://www.aphis.usda.gov/newsroom/hot_issues/bse/surveillance/ongoing_surv_results.shtml

P02.35

Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK-resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf

Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-086-05 Project Type: Specific Cooperative Agreement

Start Date: Sep 15, 2004 End Date: Sep 14, 2009

Objective: The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.

Approach: This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.

http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490

Wednesday, February 11, 2009

Atypical BSE North America Update February 2009

Both of the BSE cases ascertained in the US native-born cattle were atypical cases (H-type), which contributed to the initial ambiguity of the diagnosis. 174, 185 In Canada, there have been 2 atypical BSE cases in addition to the 14 cases of the classic UK strain of BSE2: one was the H-type, and the other was of the L-type.198

snip...end

source :

Enhanced Abstract Journal of the American Veterinary Medical Association January 1, 2009, Vol. 234, No. 1, Pages 59-72

Bovine spongiform encephalopathy

Jane L. Harman, DVM, PhD; Christopher J. Silva, PhD

http://avmajournals.avma.org/doi/ref/10.2460/javma.234.1.59

Atypical BSE North America Update February 2009

http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html

Sunday, May 17, 2009

WHO WILL WATCH THE CHILDREN ? SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

http://downercattle.blogspot.com/2009/05/who-will-watch-children.html

http://downercattle.blogspot.com/

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Sunday, September 6, 2009

MAD COW USA 1997 SECRET VIDEO

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html

DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN SEE VIDEO

http://maddeer.org/video/embedded/prusinerclip.html

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html

Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html

Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html

Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ? August 20, 2008

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html

QFC s Delayed Mad Cow Response Draws Lawsuit

Family claims QFC should have used customer database to warn those at risk sooner

March 05, 2004

SEATTLE A Bellevue, Wash. family today filed a proposed class-action lawsuit against Quality Food Centers (QFC), a subsidiary of Kroger (NYSE: KR), claiming the grocery store chain should have used information gathered through its customer loyalty program to warn those who purchased beef potentially tainted with mad cow disease.

The suit, filed in King County Superior Court, seeks to represent all Washington residents who purchased the potentially tainted meat, and asks the court to establish a medical monitoring fund.

Jill Crowson purchased the potentially tainted beef from a Bellevue QFC on Dec. 22 and 23, and used her Advantage Card, QFC s customer loyalty program. She served the meat to her husband over Dec. 25 and 26, and later heard of the recall in the newspaper.

Steve Berman, the attorney representing the Crowsons, asserts that since the company tracks purchases, it should have warned the Crowsons and many other customers who purchased the beef at approximately 40 stores across Washington.

If you lose your keys with an Advantage Card attached, QFC will return them to you free of charge, said Berman. If they can contact you over a lost set of car keys, why couldn t they contact you and tell you that the beef you purchased could kill you?

QFC is among the large number of grocers that track customer purchases through loyalty cards like the Advantage Card. Once a customer shares contact information including name, address and phone number they are given discounts on certain items.

Regardless of any discounts offered, the loyalty card tracks customers every purchase and stores them in a central database, the complaint states.

We contend that QFC knew which Advantage Card customers purchased the suspect meat, and could have easily called to warn them, said Berman. Instead, QFC used a series of spurious excuses to hide their failure to act.

On Dec. 23, the U.S. Department of Agriculture ordered the recall of approximately 10,410 pounds of raw beef that may have been infected with bovine spongiform encephalopathy (BSE), which if consumed by humans can lead to the always-fatal Cruetzfeldt-Jakobs Disease (vCJD).

According to the complaint, QFC at first mistakenly believed it did not have any of the affected beef and took no action to remove the product from its shelves. The store later removed the beef on Dec. 24, but then did little to warn those who earlier purchased the meat, the suit claims.

It wasn t until Dec. 27 that the grocery chain posted small signs with information about the recall, the complaint alleges.

The Crowsons contacted QFC when they suspected they had purchased the potentially tainted meat, but QFC would not confirm their suspicions for two more weeks, the suit states. According to Berman, the family had to file a written request before QFC would confirm their fears.

According to health experts, Cruetzfeldt-Jakobs Disease can have an incubation period of as long as 30 years. There is no test to determine if infection took place after possible exposure, nor is there any treatment once one is infected. The condition is always fatal.

If the court grants the suit class-action status, QFC would likely be compelled to turn over the names of those who purchased the potentially tainted beef.

The proposed class-action claims QFC violated provisions of the Washington Product Liability Act by failing to give adequate warning to consumers about the potentially dangerous meat.

The suit seeks unspecified damages for the plaintiffs, as well as the establishment of a medical monitoring fund.

http://www.hagens-berman.com/frontend?command=PressRelease&task=viewPressReleaseDetail&iPressReleaseId=654

QFC's Delayed Mad Cow Response Draws Lawsuit

... subsidiary of Kroger , claiming the grocery store chain should ... beef potentially tainted with "mad cow disease ...
beef at approximately 40 stores across Washington. ...
www.forrelease.com/D20040305/sff005.P2.03042004214558.03634.html - 9k -

040307 Woman Sues QFC Over Mad-Cow Recall ...
Jakob disease, the human form of mad-cow, from eating ...
QFC is subject to the Washington Product Liability ...
been found in a slaughtered Yakima County dairy cow. ...
www.spcnetwork.com/mii/2004/040307.htm - 6k

SUPERIOR COURT OF THE STATE OF WASHINGTON FOR KING COUNTY

JILL CROWSON, ET AL., PLAINTIFFS

VS

QUALITY FOOD CENTERS, INC., an Ohio corporation Defendent

NO. 04-2-05608-0 SEA

snip...

The Court hereby GRANTS the defendant's motion to dismiss the plaintiff's claims based on a manufacturer's strict liability (Counts I and II) and DENIES the defendant's motion to dismiss the plaintiff's claim of negligence by a product seller (Count III).

DATED this 14th day of June, 2004

snip...

http://www.hagens-berman.com/files/Mad%20Cow%20Order%20Denying%20Motion%20to%20Dismiss1088546283878.pdf

Date Filed: March 5, 2004 Court: King County Superior Court (Washington) Location: Seattle Ticker Symbol: NYSE:KR

Join This Suit Tell a Friend

Consumers filed a proposed class-action lawsuit against Quality Food Centers (QFC), a subsidiary of Kroger (NYSE: KR), claiming the grocery store chain should have used information gathered through its customer loyalty program to warn those who purchased beef potentially tainted with ?mad cow disease.? The USDA issued a recall notice for the meat on December 23, 2003. QFC sold the meat through its approximately 40 stores across Washington.

The suit claims that even though QFC had the ability to quickly warn every customer who purchased the potentially deadly meat if they used the QFC Advantage Card at the time of purchase, the grocery store neglected to do so.

The suit seeks to represent every consumer in Washington state who purchased the recalled meat from QFC.

Recent Updates

June 14, 2004 - the King County Superior Court gave the green light to a suit claiming QFC didn't do enough to warn customers about beef potentially tainted with 'mad cow disease,' finding enough questions about the beef and QFC's responsibility to explore in the courtroom.

Read the court order.

http://www.hagens-berman.com/frontend?command=Lawsuit&task=viewLawsuitDetail&iLawsuitId=653

see full text ;

Tuesday, December 15, 2009 NAIS, COOL, FROM FARM TO FORK, MAD COW DISEASE

http://naiscoolyes.blogspot.com/2009/12/nais-cool-from-farm-to-fork-mad-cow.html

AS far as human transmission for CWD, you will just have to make your own minds up on that. In my opinion, there is as much evidence for transmission of cwd to humans, as there is for scrapie and BSE to humans. it's the friendly fire there from i.e. cwd exposure that concerns me the most, but the did not recall all this cwd positive elk meat FOR THE WELL BEING OF THE DEAD ELK ;

Wednesday, March 18, 2009

Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

please see ;

RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

___________________________________

PRODUCT a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;

b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;

c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;

d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;

e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;

f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9; CODE Elk Meats with production dates of December 29, 30, and 31 RECALLING FIRM/MANUFACTURER Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009. Manufacturer: Noah’s Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing. REASON Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD). VOLUME OF PRODUCT IN COMMERCE Unknown DISTRIBUTION NV, CA, TX, CO, NY, UT, FL, OK

___________________________________

END OF ENFORCEMENT REPORT FOR March 18, 2009

###

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm154840.htm

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Potential Venison Exposure Among FoodNet Population Survey Respondents, 2006-2007

Ryan A. Maddox1*, Joseph Y. Abrams1, Robert C. Holman1, Lawrence B. Schonberger1, Ermias D. Belay1 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vector-Borne, and Enteric Diseases, Centers for Disease Control and Prevention, Atlanta, GA *Corresponding author e-mail: rmaddox@cdc.gov

The foodborne transmission of bovine spongiform encephalopathy to humans, resulting in variant Creutzfeldt-Jakob disease, indicates that humans can be susceptible to animal prion diseases. However, it is not known whether foodborne exposure to the agent causing chronic wasting disease (CWD) in cervids can cause human disease. The United States Foodborne Diseases Active Surveillance Network (FoodNet) conducts surveillance for foodborne diseases through an extensive survey administered to respondents in selected states. To describe the frequency of deer and elk hunting and venison consumption, five questions were included in the 2006-2007 FoodNet survey. This survey included 17,372 respondents in ten states: California, Colorado, Connecticut, Georgia, Maryland, Minnesota, New Mexico, New York, Oregon, and Tennessee. Of these respondents, 3,220 (18.5%) reported ever hunting deer or elk, with 217 (1.3%) reporting hunting in a CWD-endemic area (northeastern Colorado, southeastern Wyoming, and southwestern Nebraska). Of the 217 CWD-endemic area hunters, 74 (34.1%) were residents of Colorado. Respondents reporting hunting were significantly more likely to be male than female (prevalence ratio: 3.3, 95% confidence interval: 3.1-3.6) and, in general, older respondents were significantly more likely to report hunting than younger respondents. Venison consumption was reported by more than half (67.4%) of the study population, and most venison consumers (94.1%) reported that at least half of their venison came from the wild. However, more than half (59.1%) of the consumers reported eating venison only one to five times in their life or only once or twice a year. These findings indicate that a high percentage of the United States population engages in hunting and/or venison consumption. If CWD continues to spread to more areas across the country, a substantial number of people could potentially be exposed to the infectious agent.

http://www.cwd-info.org/pdf/3rd_CWD_Symposium_utah.pdf

Tuesday, August 04, 2009

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

SNIP...

Thursday, April 03, 2008

A prion disease of cervids: Chronic wasting disease

2008 1: Vet Res. 2008 Apr 3;39(4):41

A prion disease of cervids: Chronic wasting disease

Sigurdson CJ.

snip...

*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

snip...

full text ;

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

From: TSS (216-119-163-189.ipset45.wt.net)

Subject: CWD aka MAD DEER/ELK TO HUMANS ???

Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"

To:

Cc: "Race, Richard (NIH)" ; ; "Belay,

Ermias"

Sent: Monday, September 30, 2002 9:22 AM

Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam,

In the Archives of Neurology you quoted (the abstract of which was

attached to your email), we did not say CWD in humans will present like

variant CJD.

That assumption would be wrong. I encourage you to read the whole

article and call me if you have questions or need more clarification

(phone: 404-639-3091). Also, we do not claim that "no-one has ever been

infected with prion disease from eating venison." Our conclusion stating

that we found no strong evidence of CWD transmission to humans in the

article you quoted or in any other forum is limited to the patients we

investigated.

Ermias Belay, M.D.

Centers for Disease Control and Prevention

-----Original Message-----

From:

Sent: Sunday, September 29, 2002 10:15 AM

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG

HUNTERS

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

also,

A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most

serious because of rapid horizontal spread and higher prevalence than BSE in

UK, up to 15% in some populations. Also may be a risk to humans - evidence

that it is not dangerous to humans is thin.

http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summar

SEE FULL TEXT ;

Tuesday, August 04, 2009 Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/08/susceptibilities-of-nonhuman-primates.html

Sunday, April 12, 2009

CWD UPDATE Infection Studies in Two Species of Non-Human Primates and one Environmental reservoir infectivity study and evidence of two strains

http://chronic-wasting-disease.blogspot.com/2009/04/cwd-update-infection-studies-in-two.html

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. BSE was not reported in the USA.

snip...

CWD occurred principally in two locations, this one at Sybille and in a similar faccility at Fort Collins, Colorado, some 120 miles southwest. It was estimated that in total probably 60-70 cases of CWD have occurred.

It was difficult to gain a clear account of incidence and temporal sequence of events (-this presumably is data awaiting publication - see below) but during the period 1981-1984, 10-15 cases occurred at the Sybille facility.

The moribidity amongst mule deer in the facilities ie. those of the natural potentially exposed group has been about 90% with 100% mortality.

snip...

Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.

see full text 33 pages ;

http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

http://chronic-wasting-disease.blogspot.com/

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

FINAL REPORT OF A MISSION CARRIED OUT IN PORTUGAL FROM 11 TO 20 MAY 2009 IN ORDER TO EVALUATE MEASURES CONCERNING BOVINE SPONGIFORM ENCEPHALOPATHY

2009-12-03T11:12:00.000-08:00

FINAL REPORT OF A MISSION CARRIED OUT IN PORTUGAL FROM 11 TO 20 MAY 2009 IN ORDER TO EVALUATE MEASURES CONCERNING BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) AND ORGANIC FERTILIZERS AND SOIL IMPROVERS

Executive Summary

This report describes the outcome of a Food and Veterinary Office (FVO)specific audit carried out from 11 to20 May 2009, as part of the general audit of Portugal carried out under the provisions of Regulation (EC) No 882/2004 of the European Parliament and the Council.

The objective of the mission was to evaluate the implementation of certain protective measures against Bovine Spongiform Encephalopathy (BSE), as well as rules concerning organic fertilisers and soil improvers (OF/SI).

In terms of scope, the mission concentrated on BSE epidemio-surveillance in bovines, measures taken after suspicion/confirmation of BSE, removal and handling of specified risk material (SRM) from bovines, and the control measures in place to ensure the effectiveness of the total feed ban, in particular how the risks posed by the use of OF/SI are considered for the organisation of these controls. In addition and for OF/SI, it was assess the capability of the authorities to their correct production, flow and use. The evaluation included measures taken in response to the recommendations made in previous FVO missions regarding the afore-mentioned issues.

Overall, the report concludes that:

- BSE monitoring was largely satisfactory, with the exception of fallen animals, an important number of which are still not sampled and tested. However, in two cases there were significant delays in the implementation of movement restrictions following the detection of suspects. SRM controls were largely satisfactory.

- Progress has been made since the previous mission concerning feed ban controls and targets set by the control programme have been met; however, controls did not yet cover the entire country, and they were affected by deficiencies in the design and implementation of a risk based approach.

- Progress has been also made concerning OF/SI, although there were some weakness in their production. Nevertheless, official controls on the use of OF/SI have been satisfactorily reinforced, although they did not cover yet the use of non-bulk OF/SI.

The report makes a number of recommendations addressed to the Portuguese competent authorities, aimed at rectifying the shortcomings identified and further enhancing the implementing and control measures in place.

SNIP...

6 OVERALL CONCLUSIONS

BSE monitoring was largely satisfactory, with the exception of fallen animals, an important number of which are still not sampled and tested. However, in two cases there were significant delays in the implementation of movement restrictions following the detection of suspects. SRM controls were largely satisfactory.

Progress has been made since the previous mission concerning feed ban controls and targets set by the control programme have been met; however, controls did not yet cover the entire country, and they were affected by deficiencies in the design and implementation of a risk based approach. Progress has been also made concerning OF/SI, although there were some weakness in their production. Nevertheless, official controls on the use of OF/SI have been satisfactorily reinforced, although they did not cover yet the use of non-bulk OF/SI.

SNIP...END...SEE FULL TEXT ;

http://ec.europa.eu/food/fvo/act_getPDF.cfm?PDF_ID=7854

http://ec.europa.eu/food/fvo/ir_search_en.cfm?stype=insp_nbr&showResults=Y&REP_INSPECTION_REF=2009-8090

- To see the Competent Authority comments on the Draft Report, click here (89Kb) -

http://ec.europa.eu/food/fvo/act_getPDFannx.cfm?ANX_ID=6158

To see the Competent Authority response to the report recommendations, click here

http://ec.europa.eu/food/fvo/ap/ap_pt_2009-8090.pdf

Opinion of the Scientific Panel on biological hazards (BIOHAZ) on the determination of the BSE risk status of Portugal Question number: EFSA-Q-2003-093

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1178620777374.htm

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/summaryopinionportugalbse1,0.pdf?ssbinary=true

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/finalopinionportugalbse1,3.pdf?ssbinary=true

Annex to The EFSA Journal (2004) 143 on the opinion on the determination of the
BSE risk status of Portugal.

http://www.efsa.europa.eu/cs/BlobServer/Scientific_Opinion/finalreportportugalbse1,3.pdf?ssbinary=true

Update of the GBR-opinion of the SSC of July 2000 11 January 2001 - 9 - ANNEX: OVERVIEW TABLE OF ALL COUNTRIES WITH A GBR CLASSIFICATION

40 Portugal (mainland) 3/3/99 IV 2000

http://ec.europa.eu/food/fs/sc/ssc/out243_en.pdf

Wednesday, December 23, 2009

Variant Creutzfeldt–Jakob disease: the first confirmed case from Portugal shows early onset, long duration and unusual pathology

http://creutzfeldt-jakob-disease.blogspot.com/2009/12/variant-creutzfeldtjakob-disease-first.html

Table 3. Results of the GBR assessments through 2005

GBR I: Highly unlikely

Argentina (I), Australia (I), Iceland, New Caledonia, New Zealand (I), Panama (I), Paraguay (I), Singapore, Uruguay (I), Vanuatu

GBR II: Unlikely but not excluded

Botswana (I), Brazil (I), Colombia, Costa Rica (II), El Salvador (I), India, Kenya, Mauritius, Namibia (I), Nicaragua (I), Nigeria, Norway (I), Pakistan, Sweden (II). Swaziland (I)

GBR III: Likely but not confirmed or confirmed at a lower level A

lbania, Andorra, Austria, Belarus, Belgium, Bulgaria, Chile (I), Croatia, Denmark, Canada (II), Cyprus, Czech Republic, Estonia, Finland, Former Yugoslav Republic of Macedonia, France, Germany, Greece, Hungary, Ireland, Israel, Italy, Latvia, Lithuania, Luxembourg, Malta, Mexico, Poland, The Netherlands, Romania, San Marino, Slovak Republic, Slovenia, South Africa, Spain, Switzerland, Turkey, USA (II)

GBR IV: Confirmed at a higher level

Portugal, United Kingdom

ftp://ftp.fao.org/docrep/fao/010/a1000e/a1000e00.pdf

Tuesday, December 1, 2009

IMPORTATION OF CANADIAN CATTLE, BISON, SHEEP, AND GOATS INTO THE UNITED STATES 12/1/09

http://usdameatexport.blogspot.com/2009/12/importation-of-canadian-cattle-bison.html

Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html

Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html

Wednesday, November 18, 2009

R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission

http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html

Monday, November 16, 2009

CANADA, USA, specified risk materials (SRMs), Environment, Fertilizer, AND Politics, just more BSe

http://madcowspontaneousnot.blogspot.com/2009/11/canada-usa-specified-risk-materials.html

O.I.E. = B.S.E./T.S.E. ... TSS

BSE/vCJD: The European On-going Story

Prof J Ralph Blanchfield, MBE

Past President

Institute of Food Science & Technology

President 2006-2008

International Academy of Food Science & Technology

IUFoST Governing Council Member 2003-2008

Food science, food technology and food law consultant

E-mail: jralphb@easynet.co.uk Web: www.jralphb.co.uk

(updated 25 Sepember 2009)

SNIP...

BSE/vCJD

Susceptibility to vCJD (cont)

Wadsworth et al have reported that in transgenic mice expressing human PrP, human PrP 129 valine appears not to be a compatible substrate for the type of prion (type 4) seen in vCJD. These animal models suggest that human infection with BSE-derived prions may not be restricted to a single disease phenotype, but may result in sporadic CJD-like or novel phenotypes in addition to vCJD, with the type of disease experienced depending on the genotype of the host source of the infection, and the genotype of the recipient.

(Wadsworth JD et al (2004). Science 2004 Nov 11 2004)

vCJD

Asante et al have shown that transgenic mice expressing human PrP methionine 129, inoculated with either BSE or vCJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one SE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

Asante et al (2002). EMBO Journal, 21 ( 23), 6358-6366.

vCJD

First CJD case of valine homozygosity at codon 129

In a paper describing the histopathologic and molecular investigation in a young British woman with atypical sporadic CJD and valine homozygosity at PRNP codon 129. autopsy findings were atypical of sporadic CJD, with marked gray and white matter degeneration and widespread prion protein (PrP) deposition. Lymphoreticular tissue was not available for analysis. Molecular analysis of PrPSc from cerebellar tissue demonstrated a novel PrPSc type similar to that seen in vCJD (PrPSc type 4). Further studies will be required to characterize the prion strain seen in this patient and to investigate its etiologic relationship with BSE.

Mead s et al Arch Neurol. 2007;64(12):1780-1784.

vCJD

Aguzzi’s group in Zurich studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD. Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation

The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD classifications.

Polymenidou et al (2005), “Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease”, Lancet Neurology, (4):805-814

SNIP...

http://www.jralphb.co.uk/BSE_vCJD_Web.ppt

http://74.125.47.132/search?q=cache:uonjAYkres0J:www.jralphb.co.uk/BSE_vCJD_Web.ppt+DETWILER+PORTUGAL+BSE&cd=15&hl=en&ct=clnk&gl=us

Wednesday, September 9, 2009

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.

http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html

Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html

Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

Wednesday, February 11, 2009

Atypical BSE North America Update February 2009

http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html

Friday, September 4, 2009

FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009

http://madcowfeed.blogspot.com/2009/09/foia-request-on-feed-recall-product.html

Saturday, August 29, 2009

FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009

http://madcowfeed.blogspot.com/2009/08/foia-request-feed-recall-2009-product.html

Thursday, November 05, 2009 9:25 PM Subject: [BSE-L] re-FOIA REQUEST ON FEED RECALL PRODUCT contaminated with prohibited material Recall # V-258-2009 and Recall # V-256-2009

http://madcowfeed.blogspot.com/2009/11/re-foia-request-on-feed-recall-product.html

TSS

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

2009-11-23T08:50:00.000-08:00

COMMISSION DECISION of 11 November 2009

amending the Annex to Decision 2007/453/EC as regards the BSE status of Chile, Colombia and Japan
(notified under document C(2009) 8590)

(Text with EEA relevance)

(2009/830/EC)

THE COMMISSION OF THE EUROPEAN COMMUNITIES,

Having regard to the Treaty establishing the European Community,

Having regard to Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies ( 1 ), and in particular the third subparagraph of Article 5(2) thereof,

Whereas:

(1) Regulation (EC) No 999/2001 lays down rules for the prevention, control and eradication of transmissible spongiform encephalopathies (TSEs) in animals. For that purpose, the bovine spongiform encephalopathy (BSE) status of Member States or third countries or regions thereof (countries or regions) is to be determined by classification into one of three categories depending on the BSE risk involved, namely a negligible BSE risk, a controlled BSE risk and an undetermined BSE risk.

(2) The Annex to Commission Decision 2007/453/EC of 29 June 2007 establishing the BSE status of Member States or third countries or regions thereof according to their BSE risk ( 2 ) lists countries or regions according to their BSE risk status.

(3) The World Organisation for Animal Health (OIE) plays a leading role in the categorisation of countries or regions according to their BSE risk. The list in the Annex to Decision 2007/453/EC takes account of Resolution No XXI — Recognition of the Bovine Spongiform Encephalopathy

Status of Members — adopted by the OIE in May 2008 regarding the BSE status of Member States and third countries.

(4) Decision 2007/453/EC currently lists Finland and Sweden as having a negligible BSE risk and all other Member States as having a controlled BSE risk. It also lists the BSE status of third countries. In May 2009, the OIE adopted Resolution No XXII — Recognition of the Bovine Spongiform Encephalopathy Risk Status of Members. That Resolution recognised Chile as having a negligible BSE risk and Colombia and Japan as having a controlled BSE risk. The list in Decision 2007/453/EC should therefore be amended to be brought into line with that Resolution as regards those three third countries. However, pending a final conclusion of the OIE on the BSE risk status of all Member States and taking into account the harmonised stringent BSE protective measures applied within the Community, no changes should at present be made as regards the recognised BSE status of the Member States.

(5) Decision 2007/453/EC should therefore be amended accordingly.

(5) Decision 2007/453/EC should therefore be amended accordingly.

(6) The measures provided for in this Decision are in accordance with the opinion of the Standing Committee on the Food Chain and Animal Health,

HAS ADOPTED THIS DECISION:

Article 1

The Annex to Decision 2007/453/EC is replaced by the text in the Annex to this Decision.

Article 2

This Decision is addressed to the Member States.

Done at Brussels, 11 November 2009.

For the Commission
Androulla VASSILIOU
Member of the Commission

ANNEX

‘LIST OF COUNTRIES OR REGIONS

A. Countries or regions with a negligible BSE risk
Member States

— Finland,
— Sweden,

EFTA countries

— Iceland,
— Norway,

Third countries

— Argentina,
— Australia,
— Chile,
— New Zealand,
— Paraguay,
— Singapore,
— Uruguay,

B. Countries or regions with a controlled BSE risk
Member States

— Belgium, Bulgaria, the Czech Republic, Denmark, Germany, Estonia, Ireland, Greece, Spain, France, Italy, Cyprus, Latvia, Lithuania, Luxembourg, Hungary, Malta, Netherlands, Austria, Poland, Portugal, Romania, Slovenia, Slovakia, United Kingdom,

EFTA countries

— Liechtenstein,
— Switzerland,

Third countries

— Brazil,
— Canada,
— Colombia,
— Japan,
— Mexico,
— Taiwan,
— United States,

C. Countries or regions with an undetermined BSE risk

—

http://eur-lex.europa.eu/LexUriServ/LexUriServ.do?uri=OJ:L:2009:295:0011:0013:EN:PDF

bought and paid for by your local cattle dealers. ...TSS

IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

http://www.oie.int/eng/Session2007/RF2006.pdf

Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801e47e1

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028 Public Submission Title Comment from Terry S Singletary

Comment 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.

MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure. ...

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8151

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8

Wednesday, November 18, 2009

R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission

http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html

Tuesday, November 10, 2009

Surveillance On the Bovine Spongiform Encephalopathy and rabies in Taiwan

http://usdavskorea.blogspot.com/2009/11/surveillance-on-bovine-spongiform.html

Monday, October 26, 2009

MAD COW DISEASE, AND U.S. BEEF TRADE

MAD COW DISEASE, CJD, TSE, SOUND SCIENCE, COMMERCE, AND SELLING YOUR SOUL TO THE DEVIL

http://usdameatexport.blogspot.com/2009/10/mad-cow-disease-and-us-beef-trade.html

Posted: Wed Dec 19, 2007 3:47 pm Post subject: OIE

--------------------------------------------------------------------------------

Question wrote:

Quote:
Maybe familirise yourself with the OIE. The primary concern is animal health of the world they are the animal version of the WHO. It is a long way down from that ivory tower but here we go, until pressured by the USA repesentatives a country could not export animals for 6 years after finding a BSE/BASE positive animal so under the old rules the US would not be able to export anywhere in the world for another 4 1/2 years. Who got the risk levels system put in to allow some trade - your US representatives. You guys want to change rules - OK , but you do not get special rules that only apply to the US.

As i have told you before Sand h I market all my own slaughter animals and you know that, so don't do the whole holier than thow act.

With all due respect, it is obvious that you know little about the OIE and how it actually works. Having been to their offices in Paris and talked personally with the Head of the Animal Test Section, you would choke if you knew how many lobby groups attend that office daily. There is a steady stream of paid lobby groups that have one goal in life and that is to sway the Section Heads of each department within the OIE to suit the needs of different juristictions around the world, which curiously enough, also includes the USA and Canada. Anyone can go there and chat with them - providing they can privide valid cause to be let in. To say that the only goal of the OIE is animal health is actually only part of their function. They are more than that and my discussions with Dr. Diaz there has showed me that. But to blindly make a statement regarding what they do when you have no idea what they actually do is like eating the skin of the orange and not knowing what is actually under.

Interstingly you state that the US Government applied pressure (to the OIE) I assume and that is a great example of the lobby groups doing their job. So, at the end of the day, one can safely assume that it is the pressure applied by certain influential lobby groups that will determine a likely aoutcome to an apparent OIE directive. Man alive, isn't it great to live in a democracy wherein the people get to make the choices and not just some "other" interested party or group - say like........Cargyll or Tyson for example?

So, one last question, question?

Who wags the tail of that dog?? And for what reason other than one that is purely associated with trade and international agreements and greed?

And you think it is so simply explainable.

http://www.ranchers.net/forum/viewtopic.php?t=22833&postdays=0&postorder=asc&highlight=ops&start=36

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of the United States of America (USA) Question number: EFSA-Q-2003-083 Adopted date: 1 July 2004 Summary (0.1Mb)

Document (0.2Mb)

Summary

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.

A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1178620779461.htm

http://www.efsa.europa.eu/EFSA/Scientific_Document/sr03_biohaz02_usa_report_annex_en1.pdf?ssbinary=true

http://www.efsa.europa.eu/EFSA/Scientific_Document/sr03_biohaz02_usa_report_v2_en1.pdf?ssbinary=true

http://www.efsa.europa.eu/EFSA/Scientific_Document/sr03_biohaz02_usa_report_summary_en1.pdf?ssbinary=true

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of CANADA

Question N° EFSA-Q-2003-083 Adopted July 2004 Summary The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC), to provide an up-to-date scientific report on the GBR in Canada, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Canada. This scientific report addresses the GBR of Canada as assessed in 2004 based on data covering the period 1980-2003. The BSE agent was probably imported into the country middle of the eighties and could have reached domestic cattle in the early nineties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early 90s. It is possible that imported meat and bone meal (MBM) into Canada reached domestic cattle and led to an internal challenge in the early 90s. A certain risk that BSE-infected cattle entered processing in Canada, and were at least partly rendered for feed, occurred in the early 1990s when cattle imported from UK in the mid 80s could have been slaughtered. This risk continued to exist, and grew significantly in the mid 90's when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries. EFSA concludes that the current GBR level of Canada is III, i.e. it is confirmed at a lower level that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as the system remains unstable, it is expected that the GBR continues to grow, even if no additional external challenges occur.

http://www.mvo.nl/wetgeving-dierlijk-vet/onderzoek/download/EFSA%20on%20BSE%20risk%20Canada%20jul%202004.pdf

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of MEXICO

Question N° EFSA-Q-2003-083 Adopted July 2004 Summary The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in Mexico, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Mexico. This scientific report addresses the GBR of Mexico as assessed in 2004 based on data covering the period 1980-2003. The BSE agent was probably imported into Mexico and could have reached domestic cattle. These cattle imported could have been rendered and therefore led to an internal challenge in the mid to late 1990's. It is possible that imported meat and bone meal (MBM) into Mexico reached domestic cattle and leads to an internal challenge around 1993. It is likely that BSE infectivity entered processing at the time of imported 'at - risk' MBM (1993) and at the time of slaughter of imported live 'at - risk' cattle (mid to late 1990s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable. Thus it is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system. EFSA concludes that the current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSEagent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.

http://www.mvo.nl/wetgeving-dierlijk-vet/onderzoek/download/EFSA%20on%20BSE%20risk%20Mexico%20jul%202004.pdf

MY comments/questions are as follows ; 1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?

*** Suppressed peer review of Harvard study October 31, 2002 ***

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf

***

http://www.scribd.com/doc/1490709/USDA-200600111

***

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

***

http://www.regulations.gov/search/Regs/contentStreamer?objectId=090000648027c28e&disposition=attachment&contentType=pdf

***

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

***

Response to Public Comments on the Harvard Risk Assessment of ... RESPONSE TO COMMENTS FROM TERRY S. SINGELTARY SR. Comment #1: SINCE the first Harvard BSE Risk Assessment was so flawed and fraught ...

http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf

Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles

Thursday, November 13, 2008

Katharina Stoeck1, 6, Klaus Hess2, Lorenz Amsler3, 7, Tobias Eckert3, Dieter Zimmermann4, Adriano Aguzzi1, 8 and Markus Glatzel5, 8

(1) Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland (2) Dept. of Neurology, University Hospital Zurich, Zurich, Switzerland (3) Swiss Federal Office of Public Health, Bern, Switzerland (4) Institute of Surgical Pathology, University Hospital Zurich, Zurich, Switzerland (5) Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany (6) Dept. of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany (7) CSL Behring, Bern, Switzerland (8) Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany

Received: 23 February 2007 Revised: 8 February 2008 Accepted: 11 February 2008 Published online: 29 October 2008

Abstract Incidences of human transmissible spongiform encephalopathies are monitored by national registries in the majority of countries in Western Europe. During the past 13 years incidences for Creutzfeldt-Jakob disease (CJD) in Switzerland fluctuated between 0.4 and 2.63 cases/106 inhabitants. We have compared clinicpathological patient profiles including geographic and gender distribution, age at disease onset, duration of disease, clinical symptoms, and recognized or hypothetical risk factors for CJD, genetic risk factors, biochemical and histopathological data for two cohorts of Swiss sporadic CJD patients from years of regular sporadic CJD incidence (1996–2000, mean incidence 1.3 cases/106 inhabitants, n = 47) to Swiss sporadic CJD patients from years of elevated sporadic CJD incidence (2001–2004, mean incidence 2.3 cases/106 inhabitants, n = 73). Sporadic CJD patients from the cohort with elevated sporadic CJD incidence presented with a higher frequency of rare sporadic CJD subtypes. Patients of these subtypes were significantly older and showed a skewed male/female ratio when compared to published patients of identical sporadic CJD-types or to patients from the 1996–2000 cohort and indicates that improved detection of rare sporadic CJD subtypes may have contributed to increased incidence.

snip...

In summary, this analysis confirms our initial finding of an increased incidence of sCJD in Switzerland. Although, the reason for this phenomenon remains unexplained to date, our analysis demonstrates that patients from the years 2001–2004 with increased sCJD incidence differ in several aspects from published sCJD cohorts. The fact that the MV2 subgroup of patients showed an increase in mean age at disease onset when compared to published cohorts, together with the fact that these patients demonstrate distinct features in sensitive imaging methods, may indicate that improved detection of these patients has contributed to the rise in sCJD incidence. Further studies investigating biochemical and genetic aspects will contribute to our understanding of the mechanisms underlying sCJD.

Electronic supplementary material The online version of this artiecle (DOI10.1007/s00415-008-0900-00) contains supplementary material, which is available to authorized users. Key words Creutzfeldt-Jakob disease - prions - dementia - epidemiology

M. Glatzel and A. Aguzzi coordinated the design and operation of the study. Katharina Stoeck and Klaus Hess were involved in clinical assessment of patients. M. Glatzel and Dieter Zimmermann were involved in assessment of specimen. All authors contributed to the manuscript and approved the final version. M. Glatzel and A. Aguzzi had full access to all data in the study and had final responsibility for the decision to submit for publication. The study was performed according to established ethical guidelines This study was supported by grants of the Swiss Federal Office of Public Health and the Swiss National Science Foundation.

http://www.springerlink.com/content/w2w3027q63351q12/fulltext.pdf

A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001–2004

Jessica Ruegger* 1 , Katharina Stoeck* 2,3 , Lorenz Amsler4,5 , Thomas Blaettler2,6 , Marcel Zwahlen7 , Adriano Aguzzi2 , Markus Glatzel2,8 , Klaus Hess1 and Tobias Eckert4,9

1Department of Neurology, University Hospital Zurich, Zurich, Switzerland

2Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland

3Department of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Hamburg, Germany

4Federal Office of Public Health, Bern, Switzerland

5CSL Behring, Bern, Switzerland

6Bristol-Myers Squibb, Wallingford, CT, USA

7Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland

8Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Hamburg, Germany

9Swiss Tropical Institute, Basel, Switzerland

author email corresponding author email* Contributed equally

BMC Public Health 2009, 9:18doi:10.1186/1471-2458-9-18

The electronic version of this article is the complete one and can be found online at:

http://www.biomedcentral.com/1471-2458/9/18

Received: 11 July 2008 Accepted: 14 January 2009 Published: 14 January 2009

© 2009 Ruegger et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background In 2001, the observed annual mortality from Creutzfeldt-Jakob disease (CJD) in Switzerland increased from less than 1.5 to 2.6 per million inhabitants. An underlying cause could not be identified.

Methods To analyse potential risk factors for sCJD in Switzerland, close relatives of 69 sCJD-patients and 224 frequency age-matched controls were interviewed in a case-control study using a standardised questionnaire. 135 potential risk factors including socio-demographics, medical history, occupation and diet were analysed by logistic regression adjusting for age, sex and education.

Results sCJD patients were more likely to have travelled abroad, worked at an animal laboratory, undergone invasive dental treatment, orthopaedic surgery, ophthalmologic surgery after 1980, regular GP visits, taken medication regularly, and consumed kidney. No differences between patients and controls were found for residency, family history, and exposure to environmental and other dietary factors.

Conclusion Although some factors were significantly more frequent among sCJD-cases, this study did not reveal specific explanations for the increased incidence of deaths due to sporadic CJD observed in Switzerland since 2001. Results have to be interpreted with caution due to multiple testing and possible recall bias in association with a long incubation period. The most plausible reason for the increase in Swiss sCJD cases after 2000 is an improved case ascertainment. Therefore, underreporting of cases might well have occurred before the year 2001, and the "real" yearly incidence of sCJD might not be lower than, but rather above 2 per million inhabitants.

http://www.biomedcentral.com/1471-2458/9/18

http://www.eurocjd.ed.ac.uk/sporadic.htm

Biochemical typing of pathological prion protein in aging cattle with BSE

Seraina Tester1 , Valerie Juillerat1 , Marcus G Doherr1 , Bianca Haase2 , Miroslaw Polak3 , Felix Ehrensperger4 , Tosso Leeb2 , Andreas Zurbriggen1 and Torsten Seuberlich1

1NeuroCenter, Reference Laboratory for TSE in animals, Department of Clinical Research and Veterinary Public Health, Vetsuisse Faculty, University of Berne, Switzerland

2Institute of Genetics, Vetsuisse Faculty, University of Berne, Switzerland

3National Veterinary Research Institute, Pulawy, Poland

4Institute of Veterinary Pathology, Vetsuisse Faculty, University of Zürich, Switzerland

author email corresponding author email

Virology Journal 2009, 6:64doi:10.1186/1743-422X-6-64

The electronic version of this article is the complete one and can be found online at:

http://www.virologyj.com/content/6/1/64

Received: 23 March 2009 Accepted: 26 May 2009 Published: 26 May 2009

© 2009 Tester et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract Background The broad enforcement of active surveillance for bovine spongiform encephalopathy (BSE) in 2000 led to the discovery of previously unnoticed, atypical BSE phenotypes in aged cattle that differed from classical BSE (C-type) in biochemical properties of the pathological prion protein. Depending on the molecular mass and the degree of glycosylation of its proteinase K resistant core fragment (PrPres), mainly determined in samples derived from the medulla oblongata, these atypical cases are currently classified into low (L)-type or high (H)-type BSE. In the present study we address the question to what extent such atypical BSE cases are part of the BSE epidemic in Switzerland.

Results To this end we analyzed the biochemical PrPres type by Western blot in a total of 33 BSE cases in cattle with a minimum age of eight years, targeting up to ten different brain regions. Our work confirmed H-type BSE in a zebu but classified all other cases as C-type BSE; indicating a very low incidence of H- and L-type BSE in Switzerland. It was documented for the first time that the biochemical PrPres type was consistent across different brain regions of aging animals with C-type and H-type BSE, i.e. independent of the neuroanatomical structure investigated.

Conclusion Taken together this study provides further characteristics of the BSE epidemic in Switzerland and generates new baseline data for the definition of C- and H-type BSE phenotypes, thereby underpinning the notion that they indeed represent distinct prion disease entities.

SNIP...

Conclusion Taken together these results indicate that the prevalence of H- and L-type BSE in Switzerland remains under the detection limit of the Swiss active surveillance program. However one H-type BSE case was identified by passive BSE surveillance and proves in principle the capacity to identify such cases in the population. Hence, the overall prevalence of atypical BSE in Switzerland appears very low and similar to what has been reported from other countries. It has been speculated and strengthened by experimental data [53,54] that atypical BSE once recycled in the cattle population was the origin of the worldwide BSE epidemic in the last 20 years. If this holds true and such cases occur spontaneously in the population, then BSE might never be completely eradicated. Furthermore, in these circumstances, it would be hazardous to relieve certain disease control measures, including the total prohibition of MBM in ruminant feed.

http://www.virologyj.com/content/6/1/64

Finally the authors consider the possibility that CJD in Switzerland is related to a prion epizootic, and pay considerable attention to this possibility since between 1995 and 1998, Switzerland reported a larger incidence of BSE than did all other continental European countries (415 cases between 1990 and 2002). Exposure to BSE-infected products might have taken place mainly before high-risk bovine food products were banned from the human food chain in 1990. However, BSE is thought to cause variant CJD [abbreviated as vCJD or CJD (new var.) in ProMED-mail] rather than sporadic CJD, yet all evidence indicates that none of the Swiss cases fulfill the diagnostic criteria of vCJD. Swiss CJD could be related to BSE only if the strain of Swiss BSE prion differs from the strain of BSE prevalent in the UK. Available data, though limited, suggest that this is not the case.

At present there is no evidence that the Swiss CJD cases might result from transmission of BSE to people after one or more serial passages through species other than cattle. Scrapie is exceedingly rare in Switzerland: only 7 cases have been reported in the past 10 years. Chronic wasting disease of deer has not been reported in Europe, although surveillance data on transmissible spongiform encephalopathies in European game are incomplete.

All recognized clinical and molecular markers combine to indicate that none of the Swiss patients developed vCJD. The authors conclude that the elucidation of the underlying chain of events is a national research priority, and may uncover previously unrecognized modes of prion infection and transmission. It remains to be seen whether this increase in the incidence of CJD in Switzerland will be sustained, or whether it represent a statistical anomaly. According to Will RG, et al. (Ann Neurol 1998; 43: 763-767) there was a doubling in the annual death rates for sporadic CJD in the United Kingdom between the 1980s and the 1990s, and similar increases in the apparent death rates for sporadic Creutzfeldt-Jakob disease had occurred in other European countries, attributable to improvement in diagnosis. - Mod.CP].................as/mpp/cp/mpp

http://www.promedmail.org/pls/otn/f?p=2400:1202:307098::NO::F2400_P1202_CHECK_DISPLAY,F2400_P1202_PUB_MAIL_ID:X,18755

Prions: Protein Aggregation and Infectious Diseases

ADRIANO AGUZZI AND ANNA MARIA CALELLA

Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland

snip...

3. Sporadic Creutzfeldt-Jakob disease Approximately 85% of all human prion diseases are sporadic forms of CJD. For sCJD, there is no association with a mutant PRNP allele, nor is there any epidemiological evidence for exposure to a TSE agent through contact with people or animals infected with TSEs. sCJD cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the size and glycoform ratio of proteaseresistant prion protein identified on western blot (type 1 or type 2) (174). Heterozygosity (Met/Val) at PrP codon 129 appears to be associated with a lower risk (378) and/or prolonged incubation time (119, 387). The lack of routine laboratory testing for preclinical diagnosis makes the search for agent sources and other risk factors extremely difficult. At present, the means of acquisition of a TSE agent in these patients remains a mystery. So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE. In humans, the peak age incidence of sporadic CJD is 55–60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

snip...

Physiol Rev • VOL 89 • OCTOBER 2009 • www.prv.org

http://physrev.physiology.org/cgi/content/abstract/89/4/1105

Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1

http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html

Tuesday, November 17, 2009

SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2

http://downercattle.blogspot.com/2009/11/seac-effect-of-age-on-pathogenesis-of.html

Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html

TSS

Bovine Spongiform Encephalopathy (BSE) Inspection 2009 Slovenia and Bulgaria

2009-06-23T19:10:00.000-07:00

Country Slovenia Inspection number 2009-8114 Title Bovine Spongiform Encephalopathy (BSE) Inspection period Jan 2009 Published 23/06/2009

6.1 OVERALL CONCLUSION

A satisfactory system is in place for the control and eradication of BSE. Epidemio-surveillance for BSE in bovines is largely in line with the requirements of Annex III to Regulations (EC) No 999/2001 and the officially reported incidence of BSE should be an accurate reflection of the true incidence of BSE. Controls on SRM are satisfactory. Total feed ban controls at feed mills and on farms in order to prevent feeding of ruminants with derogated PAO were satisfactory; however, the targeting criteria for the controls at farm level were not fully risk based in all regions.

http://ec.europa.eu/food/fvo/act_getPDF.cfm?PDF_ID=7441

response ;

http://ec.europa.eu/food/fvo/act_getPDFannx.cfm?ANX_ID=6036

Brussels, 16 May 2001

BSE: Scientists publish risk assessments for Costa Rica, Kenya, Slovenia and Romania

The Scientific Steering Committee (SSC) advising the European Commission on BSE related issues has today published its opinion on the Geographical Risk of Bovine Spongiform Encephalopathy (GBR) in Costa Rica, Kenya, Slovenia and Romania. The evaluation of the geographical risk of presence of BSE focuses on the risk for animals to incubate the disease. The Committee concludes that is highly unlikely that cattle infected with the BSE agent are present in domestic herds of Costa Rica (GBR level I). They found that this is unlikely but not excluded in the herds of Kenya and Slovenia (GBR level II) and that it is likely that BSE is present in the cattle herds of Romania (GBR level III) although this is not yet confirmed. Slovenia is the first accession country that is classified as GBR level II. All other accession countries evaluated so far have been classified at level III of Geographical BSE Risk. Similarly, all EU Member States are classified at level III except for Sweden, Finland and Austria (level II) and United Kingdom and Portugal (level IV).

The Committee found that Slovenia has since 1992 imported 2.400 live cattle notably from Germany, and imported small amounts of MBM. The Slovenian authorities have been able to trace most of these cattle imports and to demonstrate that many of them are still alive. They also showed that reasonably effective controls on the rendering of MBM were in place at least as of 1996, and probably also before that date. In addition, a first feed ban to ruminants was introduced in 1996. It is therefore regarded unlikely but not excluded that the BSE agent could have been recycled, but not amplified, in Slovenia between 1992 and January 2001, when a complete feed ban was put in place. Romania has imported higher numbers of live cattle (about 22,000 tons) and meat-and-bone-meal (about 10,000 tons) from EU countries where the presence of BSE has since been confirmed. Although risk management measures were taken as of 1996, their effective enforcement has not been demonstrated. Therefore it is regarded likely that Romanian cattle herds were exposed to potentially BSE contaminated feed and subsequently infected.

Kenya has received meat and bone meal exports notably between 1987-1990 from the UK and since 1994 from Belgium, Denmark and the Netherlands. The data made available to the SSC do not exclude that some of this MBM has reached domestic cattle. The conclusion of the assessment for Costa Rica is based on data demonstrating that BSE infectivity is highly unlikely to have reached the country and hence the domestic cattle population. Only minor quantities of potentially infected live cattle (35 from Spain) or potentially contaminated meat-and-bone meal (5 tonnes) were imported into the country.

The SSC recommends that BSE related aspects are included in the programme of future inspection missions of the Food and Veterinary Office, as far as feasible, to obtain confirmation of the information received from the national authorities in the countries concerned. For the time being, the scientists underline, their assessment has to be based on the information provided by the assessed countries. As far as possible all data have been evaluated and verified in close co-operation with the countries concerned, and checked against other sources in an open and transparent manner. Data on imports provided by the countries under evaluation have for example been compared with export data as recorded by EUROSTAT, the EU Statistical Office, and with export data provided by the UK authorities.

The evaluation of the GBR in these third countries was made on the basis of the same method and assessment process as described by the SSC in its July 2000 opinion on the GBR( 1 ). In the July-opinion the scientists already assessed the GBR risk in all EU Member States except Greece, and a first series of third countries( 2 ). An assessment for Uruguay was published in January; assessments for Botswana, Lithuania, Namibia, Nicaragua, and Swaziland in February, and for Albania, Brazil, Colombia, Republic of Cyprus, Czech Republic, Estonia, Hungary, India, Mauritius, Pakistan, Poland, Singapore and Slovakia in April this year.

The full text of the opinions is available at:

http://ec.europa.eu/food/fs/sc/ssc/outcome_en.html

Released on 29/05/2001

http://ec.europa.eu/dgs/health_consumer/library/press/press138_en.html

Bovine Spongiform Encephalopathy, Slovenia

Impact Worksheet, November 23, 2001

http://www.aphis.usda.gov/vs/ceah/cei/taf/iw_2001_files/foreign/bse_slovenia1101.htm

Country Bulgaria Inspection number 2009-8110 Title Bovine Spongiform Encephalopathy (BSE) Inspection period Feb 2009 Published 23/06/2009

6.6 OVERALL CONCLUSION

The report concludes that very little progress has been made since the previous mission concerning the monitoring of on-farm slaughtering, as a result of which requirements for epidemio-surveillance and SRM are not complied with at this level; moreover, testing of fallen animals is still limited and passive surveillance has not resulted in the declaration of any suspect so far. On the contrary, epidemio-surveillance and SRM controls at slaughterhouse level were largely satisfactory; the same applies to feed ban controls, although there were deficiencies in the organization of controls in accordance with risks. ...

http://ec.europa.eu/food/fvo/act_getPDF.cfm?PDF_ID=7439

response ;

http://ec.europa.eu/food/fvo/ap/ap_bulgaria_8110_2009.pdf

MIDDAY EXPRESS News from the Press and Communication Service's midday briefing Nouvelles du rendez-vous de midi du Service Presse et Communication 02 / 07 / 2002 EXTRAIT BSE: Scientists publish geographical risk assessments (GBR) for seven countries - Bulgaria, Croatia, Iceland, Latvia, San Marino, Turkey and Vanuatu GBR is a qualitative indicator of the likelihood of the presence of one or more cattle being infected with BSE. Where its presence is confirmed GBR gives an indication of the level of infection. The evaluation focuses on the risk for animals to incubate the disease. There are four categories: I Highly unlikely; II Unlikely but not excluded; III Likely but not confirmed or confirmed, at a lower level; IV Confirmed at a higher level. The Scientific Steering Committee which advises the European Commission on BSE related issues, has concluded that it is highly unlikely that cattle infected with the BSE agent are present in the domestic herds in Iceland and Vanuatu (GBR level I). They concluded that it is likely that BSE is present in the cattle herds of Bulgaria, Croatia, Latvia, San Marino and Turkey, although this is not yet confirmed (GBR level III). The full texts of the opinions are available

at:

http://europa.eu.int/comm/food/fs/sc/ssc/outcome_en.html#reports

http://ec.europa.eu/dgs/health_consumer/library/press/press241_en.pdf

Scientific Steering Committee June 2002 - 1 - Opinion of the Scientific Steering Committee on the GEOGRAPHICAL RISK OF BOVINE SPONGIFORM ENCEPHALOPATHY (GBR) in Bulgaria Adopted by the SSC on 27 June 2002

http://ec.europa.eu/food/fs/sc/ssc/out271b_en.pdf

Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary

snip...

your only fooling yourselves with this stupid ukbsenvcjd only theory, and the BSE methology of the OIE. most any coutnry that went by those same OIE BSE guidelines all went down with BSE.

THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE.

AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...

snip...

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801e47e1

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8

Docket APHIS-2007-0033 Docket Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Docket Type Rulemaking Document APHIS-2007-0033-0001 Document Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Public Submission APHIS-2007-0033-0002.1 Public Submission Title Attachment to Singeltary comment

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=090000648027c28e

Monday, June 01, 2009

Biochemical typing of pathological prion protein in aging cattle with BSE

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html

Sunday, June 07, 2009

L-TYPE-BSE, H-TYPE-BSE, C-TYPE-BSE, IBNC-TYPE-BSE, TME, CWD, SCRAPIE, CJD, NORTH AMERICA

http://bse-atypical.blogspot.com/2009/06/l-type-bse-h-type-bse-c-type-bse-ibnc.html

Sunday, May 10, 2009

Identification and characterization of bovine spongiform encephalopathy cases diagnosed and NOT diagnosed in the United States

http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.html

Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html

Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html

Saturday, February 28, 2009 NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009 SEAC 102/2

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html

Saturday, June 13, 2009

BSE FEED VIOLATIONS USA UPDATE From 01/01/2009 To 06/10/2009

http://madcowfeed.blogspot.com/2009/06/bse-feed-violations-usa-update-from.html

Thursday, March 19, 2009

MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA

http://madcowfeed.blogspot.com/2009/03/millions-and-millions-of-pounds-of-mad.html

WHO WILL FOLLOW THE CHILDREN FOR CJD SYMPTOMS ???

Saturday, May 2, 2009

U.S. GOVERNMENT SUES WESTLAND/HALLMARK MEAT OVER USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM

http://downercattle.blogspot.com/2009/05/us-government-sues-westlandhallmark.html

Sunday, April 12, 2009 BSE MAD COW TESTING USA 2009 FIGURES Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620

http://www.aphis.usda.gov/newsroom/hot_issues/bse/surveillance/ongoing_surv_results.shtml

SEE FULL TEXT ;

http://madcowtesting.blogspot.com/2009/04/bse-mad-cow-testing-usa-2009-figures.html

Monday, May 4, 2009

Back to the Past With New TSE Testing Agricultural Research/May-June 2009

http://madcowtesting.blogspot.com/2009/05/back-to-past-with-new-tse-testing.html

Thursday, April 9, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed

http://madcowfeed.blogspot.com/2009/04/docket-no-fda2002n0031-formerly-docket.html

http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-problem-dont.html#comments

Sunday, April 12, 2009 r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada

http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-problem-dont.html

http://prionunitusaupdate2008.blogspot.com/2009/04/cjd-foundation-sides-with-r-calfers-no.html#comments

Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

GREETINGS,

so, let us postulate shall we ;-) let us just postulate that for just this one time, that mad cow disease and all other Transmissible Spongiform Encephalopathies in all other species, that have been feeding on these species, and in the laboratory studies that proves oral transmission in many different species of these TSE, and in some the lateral and vertical transmission, let us all ignore this as well, just this one time. let's just for this one second play like the spontaneous mad cow disease is for real (which i don't believe for one second), and that mad cow disease just pops up from now and then, i believe it was guesstimated to be around to be like sporadic CJD i.e. 1-2 humans per million. but some studies suggested 3 to 8 cases of spontaneous BSE per million head of cattle, but lets just say for grins, 1-2 per million as with sporadic CJD. Therefore, if we have about 100 million cattle in the U.S., we should have 100-200 cases of BSE each year, if you consider 100 million head of cattle per year in the USA.

so, my question, WHERE ARE THESE MAD COWS AT, AND OR WHERE ARE THEY BURIED AT since that last case of mad cow disease in the USA was made public around March of 2006 ???

by what miracle and how has the USA bovine been protected from mad cow disease for so many years, decades $$$

ALL Human and Animal Transmissible Spongiform Encephalopathy, of all phenotypes, of ALL ages, in EVERY State and INTERNATIONALLY, should be made MANDATORY reportable ASAP. ...

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518