Catalytic activity:Thiol-dependent hydrolysis of ester , thioester , amide , peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal) . ,Disease:Oxidation of Met-1 , Met-6 , Met-12 , Met-124 and Met-179 to methionine sulfoxide , and oxidation of Cys-220 to cysteine sulfonic acid have been observed in brains from Alzheimer disease (AD) and Parkinson disease (PD) patients. In AD , UCHL1 was found to be associated with neurofibrillary tangles. ,Function:Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity. ,miscellaneous:In contrast to UCHL3 , does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8. ,online information:Ubiquitin carboxy-terminal hydrolase L1 entry ,PTM:O-glycosylated. ,similarity:Belongs to the peptidase C12 family. ,subunit:Homodimer. Interacts with SNCA (By similarity) . Interacts with COPS5. ,tissue specificity:Found in neuronal cell bodies and processes throughout the neocortex (at protein level) . Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. ,
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