FunctionUbiquitin-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.
Tissue specificityFound 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. Down-regulated in brains from Parkinson disease and Alzheimer disease patients.
Involvement in diseaseParkinson disease 5 Neurodegeneration with optic atrophy, childhood-onset
Sequence similaritiesBelongs to the peptidase C12 family.
Cellular localizationCytoplasm. Endoplasmic reticulum membrane. About 30% of total UCHL1 is associated with membranes in brain.