title: Pathological prion protein in muscles of hamsters and mice infected with rodent-adapted BSE or vCJD.
abstract: 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.
authors.affiliation: Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
authors.forename: Achim
authors.initials: A
authors.lastname: Thomzig
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Cardone
authors.affiliation: Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
authors.forename: Dominique
authors.initials: D
authors.lastname: Krüger
authors.affiliation: Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Pocchiari
authors.affiliation: 7815 Exeter Road, Bethesda, MD 20814, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Brown
authors.affiliation: Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Beekes
mesh_terms: 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*
chemical_list: D018620:PrPSc Proteins; D011328:Prions