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疯牛病(mad cow disease),即牛传染性海绵状脑病(bovine transmissible spongiform encephalopathy,BSE)的俗称,是一种慢性消耗性、致死性、中枢神经系统退行性疾病。疯牛病被认为与朊毒体(Prion)有关,朊毒体是由正常朊蛋白(Prion protein,或者PrPC)发生构象改变后形成的异常蛋白(PrPSc)。疯牛病的发生引起了世界各国政府和科学界的高度重视,PrP的起源及其功能研究已成为研究热点。鱼类PrP相关蛋白的研究正在展开中,由于鱼类PrP相关蛋白与朊蛋白的结构相似,鱼类感染TSE类似病存在理论上的风险。本文全面地综述了疯牛病的概况、朊毒体的特性、朊毒体与哺乳动物朊蛋白、鱼类PrP相关蛋白(PrP1、PrP2和PrP3)及鱼类其他PrP相关蛋白的研究情况,为国内水生动物PrP相关蛋白研究提供参考。
Mad cow disease, commonly known as bovine transmissible spongiform encephalopathy (BSE), is a chronic, debilitating, central nervous system degenerative disease. BSE is considered to be associated with prion, which is an abnormal protein (PrPSc) that has been conformationally altered by a normal prion protein (PrPC). The occurrence of BSE has aroused the great attention of governments and scientific circles all over the world. The research on the origin and function of PrP has become a research hotspot. Studies on the PrP-related proteins in fish are underway. Since the structure of the PrP-related proteins in fish is similar to that of prion proteins, there is a theoretical risk of similar diseases in fish infected with TSE. This review provides an overview of the general situation of BSE, the characteristics of prion, prion and mammalian prion, fish PrP related proteins (PrP1, PrP2 and PrP3) and other PrP related proteins in fish research situation for the domestic aquatic Animal PrP-related proteins for reference.