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目前已知至少有6种动物性和回种人类致命的传播性海绵样脑病(TSE),其中包括近来发现的牛海绵状脑病(BSE)和半个世纪以前发现的人类克-雅氏病(CJD)。研究表明,这些疾病的发生与朊感染媒介蛋白(PrP)有关。PrP木是机体组织中的一种正常成份(PrPc),具有重要的生理功能,PrP基因突变及其所编码的异型PrP(PrP3c)却能导致若干遗传性和感染性TSE的发生。PrP生理与病理机制的进一步认识具有重要的生物学和医学意义。
There are at least six animal-borne and recurrent human lethal transmissible spongiform encephalopathies (TSEs) known to date, including the recent discovery of bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease that was discovered half a century ago CJD). Studies have shown that the occurrence of these diseases and prion infection protein (PrP). PrP is a normal component of body tissue (PrPc) and has important physiological functions. PrP gene mutation and its encoded PrP3c can lead to several hereditary and infectious TSEs. Further understanding of the physiological and pathological mechanisms of PrP has important biological and medical implications.