機能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.
組織特異性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. Down-regulated in brains from Parkinson disease and Alzheimer disease patients.
関連疾患Parkinson disease 5 Neurodegeneration with optic atrophy, childhood-onset
配列類似性Belongs to the peptidase C12 family.
細胞内局在Cytoplasm. Endoplasmic reticulum membrane. About 30% of total UCHL1 is associated with membranes in brain.