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From: TSS ()
Subject: A special form of PrPSc in muscles of a BSE-infected cow ''PRION 2005''
Date: March 9, 2007 at 9:18 am PST

“Prion 2005: Between fundamentals and society’s needs”

Tuesday, 18.October 2005



A special form of PrPSc in muscles of a BSE-infected cow

I Schiller1, J Duss1, F Kuhn1, J Schmid1, M Glatzel2, F Ehrensperger2, M Hilbe2, K Zlinszky2, B

Stierstorfer3, B Oesch1, A.J. Raeber1

1 Prionics AG, Switzerland; 2 Universitiy of Zurich, Switzerland; 3 Ludwig-Maximilians-Universitaet Muenchen,


PrPSc, the abnormal form of the prion protein, has been detected in muscles of patients with

sporadic Creutzfeldt-Jakob disease, in natural and experimental scrapie in sheep and in ro-dent

adapted scrapie but not in cattle with bovine spongiform encephalopathy (BSE).

In this study we have analyzed 60 muscle samples originating from 7 Swiss and 6 Bavarian cows

with confirmed BSE by 3 different methods: (1) phosphotungstic acid precipitation fol-lowed by

proteinase K digestion, (2) phosphotungstic acid precipitation followed by immuno-precipitation

with the PrPSc conformation specific antibody 15B3, and (3) ultracentrifugation followed by

immunoprecipitation with the antibody 15B3. Analysis of PrP was performed by Western

blotting with the antibody 6H4. Here we report for the first time the detection of a special form of

PrPSc in muscles of a clinical BSE case. This form of PrPSc is protease-resistant yet the

protease-resistant fragment detected on Western blot differs from that typi-cally found in BSE

positive brains. In 1 of 7 Swiss BSE cases, we detected PrPSc in sem-itendinous muscle, triceps

muscle, sternocephalicus muscle and in the tongue but not in two other muscles analysed. Our

findings show that low levels of a special form of PrPSc are present in a small percentage of

muscles from BSE affected cattle. Whether this type of PrPSc in muscle of cattle with BSE is

associated with infectivity remains to be established. 227 of 411 pages...tss


Pathological Prion Protein in Muscles of Rodents Infected with BSE or vCJD

Achim Thomzig1, Franco Cardone2, Dominique Krüger1, Maurizio Pocchiari2, Paul Brown3,

Michael Beekes1

1 Robert Koch-Institut, P24; 2 Instituto Superiore di Sanitŕ, Depatment of Cell Biology and Neurosciences, Rome, Italy;

3 Bethesda, Maryland, USA

Recently, pathological prion protein PrPTSE was detected in muscles from sheep infected with

scrapie, the archetype of transmissible spongiform encephalopathies (TSEs). This finding has

highlighted the question of whether mammalian muscle may potentially also provide a reservoir

for TSE agents related to Bovine Spongiform Encephalopathy (BSE) and variant Creutzfeldt-

Jakob Disease (vCJD). We here report results from studies in hamsters and mice which provide

direct experimental evidence, for the first time, of BSE- and vCJD-associated PrPTSE deposition

in muscles. Our findings emphasize the need for further assessment of possible public health

risks from TSE involvement of skeletal muscle. 311...tss

also, see ;


Transmission of BSE to cynomolgus macaques

J Montag1, G Hunsmann1, W Schul-Schaeffer2, D Motzkus1

1 German Primate Centre, Department of immunology and virology, Göttingen, Germany; 2 Georg-August-Universität

Göttingen, Dept. of Neurology, Robert-Koch-Str. 40, 37075 Göttingen, Germany

The risk of transmitting transmissible spongiform encephalopathies (TSEs) from animal to

humans is still an important question in TSE research. There are scientific indications that bovine

spongiform encephalopathy (BSE) is transmitted to humans causing variant Creutzfeldt Jakob

disease (vCJD). Previous studies have shown that infectious material from cattle can cause TSEs

in cynomolgus macaques (M. fascicularis) resembling the lesion profile of vCJD. Recently, oral

infection using macaque-adapted brain homogenate was successful in the same animal model.

In cooperation with five European partners a quantitative study for the transmission of the BSE

agent to M. fascicularis was initiated to assess the risk of vCJD infection in humans through

contaminated of BSE-infected material. Infection was performed orally and, as a control,

intracerebrally with non adapted BSE material. As the first result we report the successful

intracerebral transmission of BSE to M. fascicularis. 6/6 have died of TSE. The analysis of one

animal is exemplified here. By immunohistology, amyloid plaques with PrP depositions were

detected in different brain sections. The Western Blot analysis revealed PrP-specific bands

between 26 and 39 kDa. After PK-digestion of the infected macaque brain homogenate this

pattern shifted to three bands between 17 - 36 kDa. The bandshift was also detected after PNGase

digestion. These data confirm the clinical diagnosis of vCJD.

page 259...tss


Adaptation of the Bovine Spongiform Encephalopathy to the Primate Microcebus

murinus by Oral Route



1 INSERM U710-EPHE-University Montpellier 2, France; 2 CNRS UPR1142, IGH, Montpellier, France; 3 INSERM

U592, Paris, FRANCE; 4 EA1784-IFR PMSE 112, University Aix -Marseille, France

Experimental transmission of BSE through oral route to mice or to non-human primates such as

Cynomolgus macaca have been reported in several studies. Here, we report on the investigation

of oral transmission of BSE to a small non-human primate, the lesser-mouse lemur (Microcebus

murinus). Seven microcebes were contaminated by bovine brain or bovine-adapted macaca brain.

Only the three microcebes that received BSE-macaca brain develop a neurological disease after

long incubation periods (44 months). The duration of clinical stage was 2 months. The symptoms

begin by nervousness evolving rapidly to agressivity, visual troubles, imbalance then

incoordination of movements, myoclonic jerks and later on, the microcebes became ataxic,

turning round on itself.

The spongiform extent was related with the duration of illness. It ranged from small discrete

vacuoles randomly dispersed throughout the neuropil to large confluent cystic spaces traversed

by thin septae. Spongiform changes were most pronounced in the thalamus, the basal ganglia, the

hypothalamus and the brainstem. The neocortex was relatively spared by spongiosis: small sparse

vacuoles were seen in the frontal and occipital lobes whereas the hippocampus showed larger

vacuoles. Spongiform changes were accompanied by important astrocytic gliosis.

Proteinase K resistant-prion protein was detected by western blot as well as

immunocytochemistry. PrPres accumulation was observed in all the microcebes presenting

clinical signs. The prion immunostaining was more intense in thalamus, basal ganglia and

brainstem. Focal deposits or plaques were seen in the cortex. In the cerebellum, some plaques

were evidenced in the molecular layer.

In conclusion, BSE agent does not seem to be directly pathogenic to microcebe by oral route, but

necessitates an adaptation of the strain to macaca. 324....tss



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