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.
Publication types
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Case Reports
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Blotting, Western
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Brain / metabolism
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Brain / pathology*
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Creutzfeldt-Jakob Syndrome / complications
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Creutzfeldt-Jakob Syndrome / metabolism
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Creutzfeldt-Jakob Syndrome / pathology*
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Humans
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Immunohistochemistry
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Male
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Muscle, Skeletal / metabolism
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Muscle, Skeletal / pathology*
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Myositis, Inclusion Body / complications
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Myositis, Inclusion Body / metabolism
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Myositis, Inclusion Body / pathology*
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Polymerase Chain Reaction
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PrPC Proteins / metabolism
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PrPSc Proteins / metabolism*
Substances
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PrPC Proteins
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PrPSc Proteins