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BSE/nvCJD The European Ongoing Story

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Sep 3, 2005
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----- Original Message -----
From: "Terry S. Singeltary Sr." <[email protected]>
To: <[email protected]>
Sent: Wednesday, November 30, 2005 3:13 PM
Subject: BSE/nvCJD The European Ongoing Story by Ralph J. Blanchfield

##################### Bovine Spongiform Encephalopathy #####################

Greetings BSE-L,

An interesting slide show by Prof Ralph J. Blanchfield. I thought some might be interested in this slide show.
Seems to be pretty up to date and thanks Ralph for adding in the part about Collinge/Asante et al and BSE to humans
as potentially being propagated as either nvCJD or sporadic CJD. ONE thing i hope that Ralph adds to his slide
show is the new study released by Aguzzi/ Polymenidou et al, because this could change many things, especially
statistics, and the study from Yokoyama and Shinagawa et al, where it showed that PrPSc was also detected
in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve), where this could change the tissues and organs
designated for SRMs, two very important studies. one other thing that might be benificial to add in the statistics,
the dramatic increase in the sporadic CJD in the USA from 1997 TO 2004. blame it on either or better surveillance (which
i could argue until that Texas mad cow that got away comes home positive;-) and or all the different TSE in species for human
animal consumption in the USA and the human TSE arising from them, via the medical/surgical/dental arena. but spontaneous
they are not. just a friendly suggestion.

kindest regards,

BSE/vCJD The European Ongoing Story

Prof J Ralph Blanchfield, MBE


Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi


Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We 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



The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species?

http://neurology.thelancet.com Published online October 31, 2005

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 [email protected]


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

e-mail; [email protected]

Symposium Secretariat

Kyomi Sasaki

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

e-mail: [email protected]


[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


Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)


Infected and Source Flocks

As of August 31, 2005, there were 115 scrapie infected and source flocks (figure 3). There were 3 new infected and source flocks reported in August (Figure 4) with a total of 148 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 102 (Figure 6), with 5 flocks released in August. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.69 :
1. In addition, as of August 31, 2005, 574 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 122 were RSSS cases (Figure 7). This includes 55 newly confirmed cases in August 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.


full text ;


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 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 here;

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 UNKNOWN???


CWD, scrapie TO HUMANS in USA = one of the six documented phenotypes of sCJD or cwdCJD or scrapieCJD ???

BSE/TSE cattle to humans in the USA = one of the six documented phenotypes of sCJD ???

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.






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