From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Monday, July 24, 2006 1:09 PM

To: FSIS RegulationsComments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

Page 1 of 98

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Greetings FSIS,

I would kindly like to comment on the following ;

[Federal Register: July 12, 2006 (Volume 71, Number 133)]

[Notices]

[Page 39282-39283]

From the Federal Register Online via GPO Access [wais.access.gpo.gov]

[DOCID:fr12jy06-35]

snip...

6. WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were

confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have ?

Imported

Belgium/Netherlands

Sheep Test Results

Background

Factsheet

Veterinary Services April 2002

APHIS

snip...

Additional tests will be conducted to determine

exactly what TSE the animals have BSE or scrapie.

These tests involve the use of bioassays that consist

of injecting mice with tissue from the infected animals

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and waiting for them to develop disease. This testing

may take at least 2 to 3 years to complete.

http://www.aphis.usda.gov/lpa/pubs/fsheet_faq_notice/fs_ahvtsheeptr.pdf

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E.

(PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES

http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&...

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E

(PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]

http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&...

> > DEPARTMENT OF AGRICULTURE

> >

> > Office of the Secretary

> >

> > [Docket No. 00-072-2]

> >

> > Declaration of Emergency Because of an Atypical Transmissible

> > Spongiform Encephalopathy (Prion Disease) of Foreign Origin

> >

> > A transmissible spongiform encephalopathy (TSE) (prion disease) of

> > foreign origin has been detected in the United States. It is different

> > from TSE's previously diagnosed in the United States. The TSE was

> > detected in the progeny of imported sheep. The imported sheep and

> > their progeny are under quarantine in Vermont. Transmissible

> > spongiform encephalopathies are degenerative fatal diseases that can

> > affect livestock. TSE's are caused by similar, as yet uncharacterized,

> > agents that usually produce spongiform changes in the brain.

> > Post-mortem analysis has indicated positive results for an atypical

> > TSE of foreign origin in four sheep in Vermont. Because of the

> > potentially serious consequences of allowing the disease to spread to

> > other livestock in the United States, it is necessary to seize and

> > dispose of those flocks of sheep in Vermont that are affected with or

> > exposed to the disease, and their germ plasm. The existence of the

> > atypical TSE of foreign origin represents a threat to U.S. livestock.

> > It constitutes a real danger to the national economy and a potential

> > serious burden on interstate and foreign commerce. APHIS has

> > insufficient funds to carry out the seizure and disposal of animals

> > and germ plasm necessary to eliminate this disease risk. These funds

> > would be used to compensate the owners of the animals and germ plasm

> > for their seizure and disposal in accordance with 21 U.S.C. 134a.

> > Therefore, in accordance with the provisions of the Act of September

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> > 25, 1981, as amended (7 U.S.C. 147b), I declare that there is an

> > emergency that threatens the livestock industry of this country and

> > hereby authorize the transfer and use of such funds as may be

> > necessary from appropriations or other funds available to agencies or

> > corporations of the United States Department of Agriculture to seize

> > and dispose of animals that are affected with or exposed to this TSE,

> > and their germplasm, in accordance with 21 U.S.C. 134a.

> >

> > Dated: This declaration of emergency shall become effective July 14,

> > 2000. Dan Glickman, Secretary of Agriculture. [FR Doc. 00-18368 Filed

> > 7-19-00; 8:45 am] BILLING CODE 3410-34-P

>

>

> >

> > I was told that ;

> >

> >

> > -------- Original Message --------

> > Subject: Re: hello Dr. Sutton...question please...scrapie...TSS

> > Date: Thu, 20 May 2004 14:36:09 -0400

> > From: Jim.D.Rogers@aphis.usda.gov

> > To: flounder@wt.net

>

>

> snip...

>

>

> FULL TEXT AND THREAD BETWEEN TSS, MAFF, USDA AND DR. DETWILER HERE ;

https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044e...?

OpenDocument

7. WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these

farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why

not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

SOME DISTURBING TSE DATA FROM BELGIUM ;

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium

B. Van Everbroeck1, A. Michotte2, R. Sciot3, C. Godfraind4, M. Deprez5, S. Quoilin6, J. -J. Martin1 and P. Cras1, 7

(1) Born-Bunge Institute (BBI), University of Antwerp (UA), Campus Drie Eiken (CDE), Antwerp, Belgium

(2) Department of Neuropathology, Academic hospital, Free University of Brussels, Brussels, Belgium

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(3) Department of Pathology, Catholic University of Leuven, Leuven, Belgium

(4) Pathology Laboratory, Catholic University of Louvain, Brussels, Belgium

(5) Laboratory of Neuropathology, University of Liège, Sart Tilman, Liège, Belgium

(6) Institute of Public Health-Louis Pasteur, Brussels, Belgium

(7) Laboratory of Neurobiology, BBI, UA, CDE, Universiteitsplein 1, B-2610 Wilrijk, Belgium

Received: 28 October 2005 Accepted: 28 March 2006 Published online: 12 July 2006

Abstract From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium.

In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain

neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In

85 patients a ‘definite’ diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained ‘probable’. We

further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat

insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72

patients different final diagnoses were made, Alzheimer’s disease being the most frequent (N = 20). The demographic

parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly

increased age-specific incidence (‰>‰6/106/year) of sCJD patients between 70 and 90 years old in the period 2002–

2004 compared to 1998–2001 and retrospectively obtained data (1990–1997, p<0.01). We undertook a detailed

clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased

vigilance for the diagnosis.

In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although

these patients are both clinically and neurobiochemically similar to the general sCJD phenotype.

Keywords Diagnosis - Epidemiology - Prion disease - Transmissible spongiform encephalopathy

http://www.springerlink.com/(tqxxirqg4xx3c4r4ay0ixz45)/app/home/contribution.asp?

referrer=parent&backto=issue,1,11;journal,1,155;linkingpublicationresults,1:102883,1

BASE in cattle in Italy of Identification of a

second bovine amyloidotic spongiform encephalopathy: Molecular

similarities with sporadic Creutzfeldt-Jakob disease

http://www.pnas.org/cgi/content/abstract/0305777101v1

Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium

H. De Bosschere, DVM, PhD

S. Roels, DVM, PhD

E. Vanopdenbosch, DVM, Lic

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Veterinary and Agrochemical Research Centre (CODA/CERVA)

National Reference Laboratorium for Veterinary TSEs

Groeselenberg 99, B-1180

Ukkel (Brussels), Belgium

KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.

ABSTRACT

For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast

to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE

strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-monthold

East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.

INTRODUCTION

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases

including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in

humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed posttranslationally

from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely

characterized, although PrPsc is believed to be its major if not unique constituent.3

Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based

on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However,

no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE

strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages

into different species, such as mice, humans, primates, and sheep.7–10 However, very recently, variant forms of BSE

have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic,

immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in

Belgium.

MATERIALS AND METHODS

Since January 2001, all cattle older than 30 months are tested for TSE via a rapid test (TeSeE-kit, Bio-Rad, Nazareth,

Belgium) after EC regulation 999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent assay

(ELISA) screening are further subjected to scrapie-associated fibrils (SAF), histopathology, immunohistochemistry,

and Western blot (WB) testing16,17 at the National Reference Laboratory (NRL).

RESULTS

A positive ELISA sample from a 64-month-old East-Flemish cow or Belgian white and red (Figure 1) was presented at

the NRL for confirmation. The animal was reported healthy before slaughter. The optical density (OD) titers at the

local laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and 0.708 (sample taken at the

contralateral side of the first sampling side of the obex region). The histopathology of the obex, pons, and midbrain

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showed no spongiform changes; immunohistochemistry of the brainstem revealed no signal of PrPsc accumulation

typical for BSE; and SAF was negative. However, WB analysis (Bovine WB, Bio-Rad, France; antibodies 12F10 and

SAF60) of the same homogenate that was prepared from the obex region for ELISA revealed a small amount of PrPsc

with an electrophoretic profile different from that of typical BSE-associated PrPsc.18,19 The band on the gel of the

non-glycosylated form of PrPsc of the present case clearly showed a lower migration pattern compared with that of a

typical BSE case (Figure 2).

DISCUSSION

For many years, researchers assumed that only one BSE strain existed.7–10 Only in the past months, reports of atypical

BSE cases were announced.11–13 The Japanese case11 describes a very young bull (23 months) characterized by the

absence of spongiform changes and PrPsc deposits immunohistochemically. The WB analysis revealed an

electrophoretic profile different from that of typical BSE, characterized by low content of the di-glycosylated molecular

form of PrPsc and a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE affected cattle with a

previously unrecognized neuropathologic profile and PrPsc type were seen. These cases were determined using a

different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on immunoblot

and a difference in regional distribution of lesions. The two cases in France13 showed variant molecular features with a

different PrPsc electrophoretic profile from other BSE cases, mainly characterized by a higher molecular mass of the

nonglycosylated PrPsc. The present case shows the most similarities (ie, identical electrophoretic profile, only ELISA

and WB positive and histopathology and immunohistochemistry negative) with the Japanese case,11 although the cow

in the Japanese case was only 23 months old, and the cow in this case was 64 months old.

The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breeddependent

feature involved. It could be that the WB techniques have become more specific within the past year in the

detection of minor differences in di-, mono-, and nonglycosylated molecular forms of PrPsc. Infection of cattle by

scrapie could also be considered since scrapie can be transmitted by direct contact between animals or through

environmental contamination.13

In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected

worldwide and may contribute to further research studies about epidemiologic significance. Current continued research

on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.

ACKNOWLEDGMENTS

The authors wish to thank Rita Geeroms, Patrick Van Muylem, Stephanie Durand, Raphaël Foubert and Amina Chama

for their technical assistance. Mario Vanpoucke is acknowledged for providing references.

REFERENCES

1. Oesch B, Westaway D, Walchii M, et al: A cellular gene encodes PrP 27–30 protein. Cell 40:735–746, 1985.

2. Prusiner SB, De Armond SJ: Prion diseases and neurodegeneration. Annu Rev Neurosci 17:311–339, 1994.

3. Prusiner SB: Scrapie prions. Annu Rev Microbiol 43:345–374, 1989.

4. Bruce M, Dickinson AG: Biological evidence that scrapie agent has an independent genome. J Gen Virol 68:79–89,

1987.

5. Fraser H, Dickinson AG: Scrapie in mice: Agent strain differences in the distribution and intensity of grey matter

vacuolation. J Comp Pathol 83:29–40, 1973.

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6. Bruce M, McConnell I, Fraser H, Dickinson AG: The disease characteristics of different strains of scrapie in Sinc

Congenic mice lines: Impications for the nature of the agent and host control of pathogenesis. J Virol 72:595–603,

1991.

7. Bruce M, Chree A, McDonnell I, et al: Transmission of bovine spongiform encephalopathy and scrapie to mice:

Strain variation and the species barrier. Philos Trans R Soc Lon Ser B 343:405–411, 1994.

8. Bruce M, Will RG, Ironside JW, et al: Transmissions to mice indicate that “new variant” CJD is caused by the BSE

agent. Nature 389:498–501, 1997.

9. Foster JD, Bruce M, McDonnell I, et al: Detection of BSE infectivity in brain and spleen of experimentally infected

sheep. Vet Rec 138:546–548, 1996.

10. Lasmezas CI, Fournier J-G, Nouvel V, et al: Adaptation of the bovine spongiform encephalopathy agent to primates

and comparison with Creutzfeldt-Jakob disease: Implications for human health. Proc Natl Acd Sci U S A 98:4142–

4147, 2001.

11. Yamakawa Y, Hagiwara K, Nohtomi K, et al, for the Expert Commitee for BSE Diagnosis, Ministry of Health,

Labour and Welfare of Japan: Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy

23-month-old Holstein steer. Jpn J Infect Dis 56:221–222, 2003.

12. Casalone C, Zanusso G, Acutis PL, et al: Identification of a novel molecular and neuropathological BSE phenotype

in Italy: International Conference on Prion Disease: from basic research to intervention concepts. Gasreig, München, 8–

10 October, 2003.

13. Biacabe AG, Laplanche JL, Ryder S, Baron T: A molecular variant of bovine spongiform encephalopathy.

International Conference on Prion Disease: From basic research to intervention concepts. Gasreig, München, 8–10

October, 2003.

14. De Becker D, Roels S, Vanopdenbosch E: BSE onderzoek: opsporen van PrPres door middel van de BIO-RAD

Platelia BSE-kit. Vlaams Diergeneeskundig Tijdschrift 69:382–384, 2000.

15. Roels S, Demeyer G, Tedik K, et al: Variance of mass (volume) taken with the calibrated syringe and of the results

provided by the Bio-Rad Platelia BSE test upon storage of brainstem samples at –20°C. Anim Res 51:493–499, 2002.

16. Roels S, De Bosschere H, Saegerman C, et al: BSE and scrapie testing in Belgium: general overview. New Food:

accepted, 2004.

17. Vanopdenbosch E, Dechamps P, Dufey J, et al: Le premier cas d’encephalopathie spongioforme bovine

diagnostique en Belgique. Annales de Médicine Vétérinaire 142:111–118, 1998.

18. Collinge J, Sidle KCL, Meads J, et al: Molecular analysis of prion strain variation and the aetiology of new variant

CJD. Nature 383:685–690, 1996.

19. Hill AF, Desbruslais M, Joiner S, et al: The same prion strain causes vCJD and BSE. Nature 389:448–450, 1997.

Figure 1. Photograph of the East-Flemish cattle breed or the Belgian white and red.

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Figure 2. Bovine Western blot (Bio-Rad, France) using antibodies 12F10 and SAF60. MM, Magic mark; Atyp. BSE,

Atypical BSE case (present case); Ref1, Reference 1 of a classical BSE case; Ref2, Reference 2 of a classical BSE

case. The third band of the non-glycosylated PrPsc of the Atyp. BSE case (left rectangle) shows a markedly faster

migration compared to the Ref1 and Ref2 cases (right rectangle).

http://www.jarvm.com/articles/Vol2Iss1/DEBOSSCHERE.htm

snip...

full text ;

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

Mad Sheep
The True Story Behind the USDA’s War on a Family Farm
Linda Faillace
The page-turning account of a government cover-up, corporate greed, and a courageous family’s fight to save their farm.

http://www.chelseagreen.com/2006/items/madsheep

got to read this months ago, and it is deeply disturbing how the feds handled this from the very beginning, and to this day we do not know the results of the mouse bio-assays, and what those sheep actually had. i don't necessarily agree with the TSE science in this book, but the book is a must read if your interested at all in human and animal TSEs. ...TSS