Ubiquitin C-terminal Hydrolases L1:Physiological and Pathological Significance / 生物化学与生物物理进展

ABSTRACT

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 from the other members of UCHs.UCH-L1 hydrolase activity could keep the pool of free ubiquitin and compromise the ubiquitin-dependent degradation pathway,while UCH-L1 dimerizational-dependent,ubiquityl ligase activity could produce undegradable,K63-linked polyubiquitin chains that could inhibit proteasomal activity.Therefore,UCH-L1 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.