Pathological prion protein in muscles of hamsters and mice infected with rodent-adapted BSE or vCJD.
Recently, pathological prion protein (PrP(TSE)) was detected in muscle 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). Here, results are reported from studies in hamsters and mice that provide direct experimental evidence, for the first time, of BSE- and vCJD-associated PrP(TSE) deposition in muscles. Our findings emphasize the need for further assessment of possible public-health risks from TSE involvement of skeletal muscle.
Thomzig
Achim
A
Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
Cardone
Franco
F
Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
Krüger
Dominique
D
Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
Pocchiari
Maurizio
M
Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
Brown
Paul
P
7815 Exeter Road, Bethesda, MD 20814, USA.
Beekes
Michael
M
Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D006224:Cricetinae; D004195:Disease Models, Animal; D016643:Encephalopathy, Bovine Spongiform / Q000473:pathology*; D051379:Mice; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D018620:PrPSc Proteins / Q000302:isolation & purification*; D017096:Prion Diseases / Q000378:metabolism / Q000473:pathology / Q000635:transmission; D011328:Prions / Q000737:chemistry / Q000235:genetics / Q000378:metabolism / Q000472:pathogenicity*
D018620:PrPSc Proteins; D011328:Prions