Ubiquitin C-terminal hydrolase L1 (UCH-L1) hydrolyze C-terminal ubiquityl esters and amides in vitro, which is an essential reaction during cytoplasmic protein degradation. peptide-ubiquityl amides are the most favorable substrates. UCH-L1 also acts as a ubiquitin (Ub) ligase. Mutation in the gene is associated with the development of neurodegenerative diseases, such as Parkinsonμs disease, spinocerebellar ataxia (SCA) and Huntingtonμs disease.
