中文摘要：

泛素C末端水解酶L1（ubiquitin carboxy-terminal hydrolases L1）属于泛素C末端水解酶家族成员,但是泛素C末端水解酶L1酶活性非常特异,不仅具有泛素C末端水解酶活性,而且具有泛素C末端聚合酶的活性.因此,泛素C末端水解酶L1,不仅在泛素化蛋白降解途径中起到关键的作用,也在其他的泛素信号途径,如在K63-多聚泛素信号途径中起重要的作用.由于泛素C末端水解酶L1特异的蛋白酶活性,也赋予了泛素C末端水解酶L1多种生物学功能,在神经发育发生、精子发生、卵子发生和受精等方面有着重要的作用.泛素C末端水解酶L1突变也与帕金森症等神经元退化疾病紧密相关.泛素C末端水解酶L1在甲状腺、肺等多种组织的超表达,也与该组织的癌症发生有着密切的联系.

英文摘要：

UCH-L1 （ubiquitin carboxy-terminal hydrolases L1） is a member of the carboxyl-terminal ubiquitin hydrolase family, naturally, that there is hydrolase activity, however, and that there is ligase acitivity, which is different fi＇om the other members of UCHs. UCH-L1 hydrolase activity could keep the pool of flee ubiquitin and compromise the ubiquitin-dependent degradation pathway, while UCH-L1 dimerizational-dependent, ubiquityl ligase activity could produce undegradable, K63-1inked polyubiquitin chains that could inhibit proteasomal activity. Therefore, UCH-LI is involved in the more diverse physiological activities, including neuron formation, gonadal development and fertilization. Mutation of UCH-L1 is linked to the neurodegenerative diseases, such as Parkinson＇s disease. Moreover, abnormal expression of UCH-L1 is response to carcinogenesis in many tissue such as thyroid lung.