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.