Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle.
case_report · Level V
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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.
Medical subject headings
- Brain
- Creutzfeldt-Jakob Syndrome
- Muscle, Skeletal
- Myositis, Inclusion Body
- PrPSc Proteins