中文摘要：

泛素C末端水解酶L1（UCH—L1）是一种在脑内高度表达，具有泛素水解酶、泛素连接酶和稳定泛素单体功能的去泛素化酶．UCH-L1参与维持神经元突触的正常形态及功能，并能够缓解β-淀粉样蛋白诱导的长时程增强（LTP）缺失及记忆障碍；此外，UCH-L1的突变体193M与家族性帕金森氏病相关，而UCH-L1的S18Y多态性则对神经元具有保护作用．通过研究UCH-L1的结构、功能及其在神经系统的作用机制，可以为阿尔茨海默病、帕金森氏病等神经退行性疾病的治疗提供相关思路和借鉴．

英文摘要：

UCH-L1 is abundantly expressed in brain which possesses ubiquitin hydrolase activity, ubiquitin ligase activity and the effect of monomeric ubiquitin stabilizing. UCH-L1 is critical for the normal morphology and function of the synapses, which can rescue the LTP deficit and impaired memory induced by β-amyloid protein （Aβ）. In addition, the I93M mutation in UCH-L1 is associated with familial Parkinson＇s disease （PD） while the S 18Y polymorphic variant of UCH-L1 is associated with a specific antioxidant protective function in neurons. By researching the structure, function and the mechanism in the nervous system of UCH-L1, hopes to provide a concept or a method to treat neurodegenerative diseases such as AD and PD.