Creutzfeldt–Jakob disease and inclusion body myositis: Abundant disease‐associated prion protein in muscle
GG Kovacs, E Lindeck‐Pozza… - Annals of Neurology …, 2004 - Wiley Online Library
GG Kovacs, E Lindeck‐Pozza, L Chimelli, AQC Araújo, AA Gabbai, T Ströbel, M Glatzel…
Annals of Neurology: Official Journal of the American Neurological …, 2004•Wiley Online LibraryPathologicalprion 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.
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.
Abstract
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.
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