朊蛋白的细胞生物学研究  

Cellular biology of prion protein

作　　者：张东威[1] 南善姬[1] 赵节绪[1] 

机构地区：[1]吉林大学第一医院神经内科,长春130021

出　　处：《生命科学》2004年第2期96-100,共5页Chinese Bulletin of Life Sciences

基　　金：国家自然科学基金项目(30070266)

摘　　要：朊蛋白病是人和牛羊等哺乳动物所患的致命性的神经系统变性疾病,它是由机体内正常的朊蛋白改变构象后所引起的疾病。本综述对朊蛋白在细胞生物学领域的认知和理解进行了归纳总结,阐述了正常和异常朊蛋白的翻译、表达、定位、裂解、转化等一系列过程,是对疾病本质的有益探索。Prion diseases are fatal neurodegenerative disorders of humans and animals that are importantbecause of their impact on public health and because they exemplify a novel mechanism of infectivity andbiological information transfer. These diseases are caused by conformational conversion of a normal hostglycoprotein (PrPC) into an infectious isoform (PrPSc) that is devoid of nucleic acid. This review focuses on thecurrent understanding of prion diseases at the cellular biological level. Information is then presented about thestructure, expression, biosynthesis, and possible function of PrPC and PrPSc, as well as its posttranslationalprocessing, cellular localization, and trafficking. The review concludes with suggestions of several importantavenues for future investigation.

关 键 词：朊蛋白 细胞生物学 朊蛋白病 

分 类 号：Q2[生物学—细胞生物学] Q51