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From: TSS ()
Subject: Research Continues Worldwide on Rare BSE Strain (10/18/06 07:35)
Date: October 19, 2006 at 10:00 am PST

Research Continues Worldwide on Rare BSE Strain (10/18/06 07:35)

OMAHA (DTN) -- Scientists know how to detect atypical BSE, but admit they have limited knowledge about the rare strain of bovine spongiform encephalopathy that has been found in the two U.S.-born cases of the disease.

A USDA scientist who conducts research on atypical BSE also declined to answer whether USDA's new testing regime would continue to find cattle infected with the atypical strain.

First discovered in France and Italy, atypical BSE has become a growing topic at scientific meetings on transmissible spongiform encephalopathies. Several research papers on the issue were presented last month at a meeting in Italy. The struggle is interpreting what atypical BSE can mean, particularly given the rarity of the disease globally.

"To be honest with you, we really don't know," said Linda Detwiler, a former USDA scientist now at the Virginia-Maryland College of Veterinary Medicine. "There's so little (data) and it's really an emerging issue right now." Scientists have found at least two strains of BSE that do not appear to show the same patterns or staining on tests as "classic BSE."

DTN first reported last May that two of the cases in the United States -- one in Texas and one in Alabama -- were atypical. A cow earlier this year in Manitoba also was an atypical case.

The two strains have been broken down into high-molecular and low-molecular strains. The high-molecular strain, which was found in the two U.S. cases, has occurred in older animals ranging from 10 to 16 years of age. When transmitting the disease to mice, the mice take longer incubation periods to show clinical signs of the disease than mice infected with classical BSE. Outside of the U.S. and Canada, the high-molecular strain has been found in six other countries.

Richt said he was confident the tests used in the U.S. can detect the diseases, even though the high-molecular BSE shows up as a weaker result on one testing protocol. In 2005, the U.S. added a second test to detect BSE cases after U.S. officials initially called the Texas case negative for BSE.

Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-073-07
Project Type: Specific C/A

Start Date: Sep 15, 2004
End Date: Sep 14, 2007

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.

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.

Subject: PrPSc distribution of a natural case of bovine spongiform encephalopathy

Date: August 8, 2005 at 12:28 pm PST

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


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


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 controversies

about definitions of SRM. Therefore we have examined PrPSc

distribution in a BSE cattle by Western blotting to reassess definitions of


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



Page 10 of 17

BSE cattle may need to be reexamined.

T. Kitamoto (Ed.)


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


"Furthermore, current studies into transmission of cases of BSE that are

atypical or that develop in young cattle are expected to amplify the BSE


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


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.

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


Symposium Secretariat

Kyomi Sasaki

TEL +81-22-717-8233 FAX +81-22-717-7656



[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle


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

1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is
with a human TSE surveillance system that is terrible
flawed. in 1997 cases of the _reported_ cases of cjd
were at 54, to 163 _reported_ cases in 2004. see stats

p.s. please note the 47 PENDING CASES to Sept. 2005

p.s. please note the 2005 Prion D. total 120(8)
8=includes 51 type pending, 1 TYPE UNKNOWN ???

p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???

p.s. please note 2004 prion disease (6) 6=7 TYPE


AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.



Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

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