NR APUX
AU Kovacs,G.G.; Lindeck-Pozza,E.; Chimelli,L.; Araujo,A.Q.; Gabbai,A.A.; Ströbel,T.; Glatzel,M.; Aguzzi,A.; Budka,H.
TI Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle.
QU Annals of Neurology 2004 Jan; 55(1): 121-5
PT case reports; journal article
AB Pathologicalprion protein (PrPsc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrPsc in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrPc-PrPsc conversion in Creutzfeldt-Jakob disease appears to become prominent when PrPc is abundantly available as substrate, as in inclusion body myositis muscle.
MH Aged; Blotting, Western; Brain/metabolism/*pathology; Comparative Study; Creutzfeldt-Jakob Syndrome/complications/metabolism/*pathology; Human; Immunohistochemistry; Male; Muscle, Skeletal/metabolism/*pathology; Myositis, Inclusion Body/complications/metabolism/*pathology; Polymerase Chain Reaction; PrPc Proteins/metabolism; PrPsc Proteins/*metabolism; Support, Non-U.S. Gov't
AD Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria.
SP englisch
PO USA