{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T19:12:38Z","timestamp":1775589158299,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Hungarian-Austrian intergovernmental S&T Cooperation Programme."}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2004,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The basis of human prion diseases affecting the nervous system is accumulation of a disease\u2010associated conformer (PrP<jats:sup>Sc<\/jats:sup>) of the normal cellular prion protein (PrP<jats:sup>C<\/jats:sup>). Earlier studies demonstrated increased expression of PrP<jats:sup>C<\/jats:sup> in inclusion body myositis (IBM), dermato\u2010, and polymyositis, as well as neurogenic muscle atrophy. To define the spectrum and reliability of PrP<jats:sup>C<\/jats:sup> immunoreactivity, its expression was examined systematically in a series of pathologically characterized muscular disorders by means of immunohistochemistry, confocal laser microscopy, and immunogold electron microscopy. Anti\u2010PrP<jats:sup>C<\/jats:sup> immunolabelling of rimmed vacuoles was observed in IBM, inclusions of myofibrillary myopathy, targets, regenerating, and atrophic fibres, mononuclear cells, in addition to ragged red fibres in mitochondrial myopathies, and focal sarcolemmal immunostaining in non\u2010diseased controls. Quantitative analysis demonstrated that, in neurogenic muscle lesions, anti\u2010PrP<jats:sup>C<\/jats:sup> staining detects a significantly broader spectrum of fibres than anti\u2010vimentin or anti\u2010NCAM. In dystrophic muscle, PrP<jats:sup>C<\/jats:sup> expression was mainly restricted to regenerating fibres. In IBM, PrP<jats:sup>C<\/jats:sup> expression was not confined to rimmed vacuoles or vacuolated fibres and only a small percentage (7.1%) of rimmed vacuoles were PrP<jats:sup>C<\/jats:sup> positive. Ultrastructurally, PrP<jats:sup>C<\/jats:sup> was observed in the cytoplasm of lymphocytes, in the myofibrillar network of targets, and in rimmed vacuoles. Knowledge of disease circumstances with altered expression of PrP<jats:sup>C<\/jats:sup> is important in the setting of a potentially increased chance for extraneural PrP<jats:sup>C<\/jats:sup>\u2013PrP<jats:sup>Sc<\/jats:sup> conversion. In addition, our observations suggest that PrP<jats:sup>C<\/jats:sup> may have a general stress\u2013response effect in various neuromuscular disorders. Copyright \u00a9 2004 Pathological Society of Great Britain and Ireland. 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