Prion Diseases and Emerging Prion Diseases

来源 :2008中国深圳蛋白质和多肽科学大会 | 被引量 : 0次 | 上传用户:xiaofeidong
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  Transmissible spongiform encephalopathies (TSES),also called prion diseases,are fatal neurodegenerative disorders.An abnormal isoform of the prion protein (PrPSc) generated by post-translational modification of the cellular prion protein (PrPC) is believed to be the main component of this infectious agent.PrPSc is relatively resistant to proteinase K (PK) digestion.This characteristic has been widely accepted as the physicochemical basis for distinguishing between PrPC and PrPSc.PrPC is a glycoprotein that contains 2 Asn-linked glycosylation sites; it is present in the cells in 3 different glycoforms,including an unglycosylated form.
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