PrP的胞内运输与朊病毒病  

The Role of Intracellular Trafficking of PrP in Prion Diseases

作　　者：王小凡[1] 韩俊[1] 高建梅[1] 万言珍[1] 李锋[1] 董小平[1] 

机构地区：[1]中国疾病预防控制中心病毒病预防控制所朊病毒室,北京100052

出　　处：《细胞生物学杂志》2005年第4期391-394,共4页Chinese Journal of Cell Biology

摘　　要：朊病毒病,即传染性海绵状脑病(transmissiblespongiformencephalopathies,TSEs),是一类致死性的神经退行性疾病,存在散发性、感染性和遗传性3种形式。在朊病毒病的病理过程中,细胞正常朊蛋白PrPC(cellularPrP)转化为异常构象的PrPSc(scrapiePrP)是至关重要的,但是朊病毒的增殖如何导致神经元凋亡仍不清楚。PrPC的胞内运输在朊病毒病中发挥重要作用,朊病毒感染后PrPC转化为PrPSc,及遗传性朊病毒病中PrP突变可能影响PrP的生物合成、亚细胞定位及转运过程,通过干扰PrPC的正常功能或产生毒性中间体而导致神经系统病变。现对近年来关于PrP胞内运输在朊病毒病中的作用进行综述。Prion diseases or transmissible spongiform encephalopathies （TSEs） are a group of fatal neurodegenerative disorders that can appear in sporadic, heritable and transmissible forms. The fundamental pathogenic mechanism of prion diseases involves the post-translational conversion of PrPc into its infectious isoform PrP^Sc, but it remains still unclear that how prion propagation leads to apoptosis of neuronal cells. Recently more evidences show that the intracellular trafficking of PrP^C may play roles in the pathogenesis of TSE. The conversion processes of PrP^C to PrP^Sc in acquired or heritable prion diseases affect biosynthesis, intracellular localization and transport of cellular PrP, leading to neurodegeneration through perturbing normal function of PrP^C or generating neurotoxic species. Possible relationship between the intracellular trafficking of PrP and molecular pathologic mechanism of prion diseases were discussed in this review.

关 键 词：朊病毒病 转化 胞内运输 PrP^Sc 运输 传染性海绵状脑病 神经退行性疾病 PrP^C 亚细胞定位 病理过程 

分 类 号：R512.99[医药卫生—内科学]