UCHL1 Antibody – Rabbit Polyclonal

The protein encoded by this gene belongs to the peptidase C12 family. This enzyme is a thiol protease that hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. This gene is specifically expressed in the neurons and in cells of the diffuse neuroendocrine system. Mutations in this gene may be associated with Parkinson disease.[provided by RefSeq, Sep 2009]

UCHL1: 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. Monomer. Homodimer. Interacts with SNCA. Interacts with COPS5. Found in neuronal cell bodies and processes throughout the neocortex. 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. Belongs to the peptidase C12 family.

Protein type: Cell development/differentiation; Protease; Ligase; EC 3.4.19.12; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 4p14

Cellular Component: nucleoplasm; endoplasmic reticulum membrane; cell soma; axon; cytoplasm; plasma membrane; cytosol

Molecular Function: ubiquitin binding; protein binding; omega peptidase activity; cysteine-type endopeptidase activity; ubiquitin-specific protease activity; alpha-2A adrenergic receptor binding; ligase activity

Biological Process: proteasomal ubiquitin-dependent protein catabolic process; cell proliferation; negative regulation of MAP kinase activity; protein deubiquitination; axon target recognition; axon transport of mitochondrion; eating behavior; neuromuscular process; muscle fiber development; sensory perception of pain; adult walking behavior

Disease: Neurodegeneration With Optic Atrophy, Childhood-onset; Parkinson Disease 5, Autosomal Dominant