Kovacs Gabor G, Lindeck-Pozza Elisabeth, Chimelli Leila, Araújo Abelardo Q C, Gabbai Alberto A, Ströbel Thomas, Glatzel Markus, Aguzzi Adriano, Budka Herbert
Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria.
Ann Neurol. 2004 Jan;55(1):121-5. doi: 10.1002/ana.10813.
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.
病理性朊病毒蛋白（PrP(Sc)）是主要影响中枢神经系统的朊病毒疾病的标志。我们通过免疫组织化学、石蜡包埋组织印迹和蛋白质印迹法，在一名散发性克雅氏病合并包涵体肌炎患者的肌肉中证实了大量PrP(Sc)的存在。在克雅氏病中，当PrP(C)作为底物大量存在时，如在包涵体肌炎肌肉中，神经外PrP(C)向PrP(Sc)的转化似乎变得显著。
