中国科技核心期刊
CN:31-1600/Q
ISSN:1004-0374
“健康与疾病的免疫”国际学术研讨会通知      关于有网站冒充本刊网站的声明
《生命科学》 2004, (2): 96-
朊蛋白的细胞生物学研究
张东威,南善姬,赵节绪*
吉林大学第一医院神经内科,长春 130021

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。
关键词:朊蛋白;细胞生物学;朊蛋白病
 
Cellular biology of prion protein
ZHANG Dong-Wei, NAN Shan-Ji, ZHAO Jie-Xu*
Department of Neurology, the First Hospital, Jilin University, Changchun 130021, China
Abstract: Prion diseases are fatal neurodegenerative disorders of humans and animals that are important because of their impact on public health and because they exemplify a novel mechanism of infectivity and biological information transfer. These diseases are caused by conformational conversion of a normal host glycoprotein (PrPC) into an infectious isoform (PrPSc) that is devoid of nucleic acid. This review focuses on the current understanding of prion diseases at the cellular biological level. Information is then presented about the structure, expression, biosynthesis, and possible function of PrPC and PrPSc, as well as its posttranslational processing, cellular localization, and trafficking. The review concludes with suggestions of several important avenues for future investigation.
Key words: prion protein; cellular biology; prion diseases
 
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