Increased Expression of the Normal Cellular Isoform of Prion Protein in Inclusion‐Body Myositis, Inflammatory Myopathies and Denervation Atrophy

The cellular isoform of the prion protein (PrPc) is a glycosylphosphatidylinositol‐anchored glycoprotein, normally expressed in neural and non‐neural tissues, including skeletal muscle. In transmissible spongiform encephalopathies, or prion diseases, PrPc, which is soluble in nondenaturing detergent and sensitive to proteinase K (PK)‐treatment, represents the molecular substrate for the production of a detergent‐insoluble and PK‐resistant isoform, termed PrPSc.

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