Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle

• Published in: Annals of neurology Vol. 55; no. 1; pp. 121 - 125
• Main Authors: Kovacs, Gabor G., Lindeck-Pozza, Elisabeth, Chimelli, Leila, Araújo, Abelardo Q. C., Gabbai, Alberto A., Ströbel, Thomas, Glatzel, Markus, Aguzzi, Adriano, Budka, Herbert
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.01.2004; Willey-Liss
• Subjects: Aged; Biological and medical sciences; Blotting, Western; Brain - metabolism; Brain - pathology; Creutzfeldt-Jakob Syndrome - complications; Creutzfeldt-Jakob Syndrome - metabolism; Creutzfeldt-Jakob Syndrome - pathology; Humans; Immunohistochemistry; Male; Medical sciences; Muscle, Skeletal - metabolism; Muscle, Skeletal - pathology; Myositis, Inclusion Body - complications; Myositis, Inclusion Body - metabolism; Myositis, Inclusion Body - pathology; Neurology; Polymerase Chain Reaction; PrPC Proteins - metabolism; PrPSc Proteins - metabolism; Infection; Striated muscle disease; Prion disease; Nervous system diseases; Central nervous system disease; Myositis; Degenerative disease; Inclusion body; Prion protein; Creutzfeldt Jakob disease; Cerebral disorder
• ISSN: 0364-5134; 1531-8249
• DOI: 10.1002/ana.10813

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.