EC Number |
Natural Substrates |
---|
3.4.19.12 | more |
enzyme inhibits cell proliferation |
3.4.19.12 | more |
isozyme L1 associates and colocalizes with monoubiquitin and elongates ubiquitin half-life, null mutants show a reduced monoubiquitin level in neurons, while overexpression causes an increase in monoubiquitin level, mechanism |
3.4.19.12 | more |
increased activity of neuronal isozyme L1 reduces the risk for Parkinson's disease |
3.4.19.12 | more |
enzyme regulation |
3.4.19.12 | more |
AMSH is a deubiquinating enzyme with functions at the endosome, it opposes the ubiquitin-dependent sorting of receptors to lysosomes, the enzyme influences the degradation of the EGF receptor by association to endosomes, model of the enzyme's physiological function |
3.4.19.12 | more |
de novo synthesis of isozyme UCH-L1 in rostral ventrolateral medulla is crucial to survival during mevinphos intoxication, the enzyme is involved in synthesis of ubiquitin which is required for protein targeting in degradation in the ubiquitin-proteasome pathway in neurons, UCH-L1 plays a neuroprotective role |
3.4.19.12 | more |
gene uch-L1 is involved in development of Parkinson's disease |
3.4.19.12 | more |
the closely related isozymes UCH-L1 and UCH-L3 function as reciprocal modulators of germ cell apoptosis in cryptorchid testis |
3.4.19.12 | more |
the enzyme is essential for the early apoptotic wave of germinal cells and for sperm quality control during spermatogenesis |
3.4.19.12 | more |
the enzyme is involved in generation of free monomeric ubiquitin, enzyme deficiency leads is involved in accumulation of ubiquitin and alpha-synuclein leading to aggregation in proteins conforming Lewy bodies and Lewy neurites leading to Parkinson's disease and dementia with Lewy bodies, the enzyme is down-regulated in Alzheimer's disease and in Parkinson's disease, comparison of enzyme expression to general protein synthesis and proteasome activity in brain |