title: Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle.
abstract: Pathologicalprion protein (PrP(Sc)) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrP(Sc) in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrP(C)-PrP(Sc) conversion in Creutzfeldt-Jakob disease appears to become prominent when PrP(C) is abundantly available as substrate, as in inclusion body myositis muscle.
authors.affiliation: Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria.
authors.forename: Gabor G
authors.initials: GG
authors.lastname: Kovacs
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Lindeck-Pozza
authors.forename: Leila
authors.initials: L
authors.lastname: Chimelli
authors.forename: Abelardo Q C
authors.initials: AQ
authors.lastname: Araújo
authors.forename: Alberto A
authors.initials: AA
authors.lastname: Gabbai
authors.forename: Thomas
authors.initials: T
authors.lastname: Ströbel
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
authors.forename: Herbert
authors.initials: H
authors.lastname: Budka
mesh_terms: D000368:Aged; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D018979:Myositis, Inclusion Body / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D016133:Polymerase Chain Reaction; D018621:PrPC Proteins / Q000378:metabolism; D018620:PrPSc Proteins / Q000378:metabolism*
chemical_list: D018621:PrPC Proteins; D018620:PrPSc Proteins