Ubiquitin carboxyl-terminal hydrolase isozyme L1 (UCH-L1) (EC 3.4.19.12) (Neuron cytoplasmic protein 9.5) (PGP 9.5) (PGP9.5) (Ubiquitin thioesterase L1)
1_MQLKP 6_ MEINP 11_ EMLNK 16_ VLSRL 21_ GVAGQ 26_ WRFVD 31_ VLGLE 36_ EESLG 41_ SVPAP 46_ ACALL 51_ LLFPL 56_ TAQHE 61_ NFRKK 66_ QIEEL 71_ KGQEV 76_ SPKVY 81_ FMKQT 86_ IGNSC 91_ GTIGL 96_ IHAVA 101_ NNQDK 106_ LGFED 111_ GSVLK 116_ QFLSE 121_ TEKMS 126_ PEDRA 131_ KCFEK 136_ NEAIQ 141_ AAHDA 146_ VAQEG 151_ QCRVD 156_ DKVNF 161_ HFILF 166_ NNVDG 171_ HLYEL 176_ DGRMP 181_ FPVNH 186_ GASSE 191_ DTLLK 196_ DAAKV 201_ CREFT 206_ EREQG 211_ EVRFS 216_AVALC
1: Deubiquitinase that plays a role in the regulation of several processes such as maintenance of synaptic function, cardiac function, inflammatory response or osteoclastogenesis (PubMed:22212137, PubMed:23359680). Abrogates the ubiquitination of multiple proteins including WWTR1/TAZ, EGFR, HIF1A and beta-site amyloid precursor protein cleaving enzyme 1/BACE1 (PubMed:22212137, PubMed:25615526). In addition, recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin to maintain a stable pool of monoubiquitin that is a key requirement for the ubiquitin-proteasome and the autophagy-lysosome pathways (PubMed:12408865, PubMed:8639624, PubMed:9774100). Regulates amyloid precursor protein/APP processing by promoting BACE1 degradation resulting in decreased amyloid beta production (PubMed:22212137). Plays a role in the immune response by regulating the ability of MHC I molecules to reach cross-presentation compartments competent for generating Ag-MHC I complexes (By similarity). Mediates the 'Lys-48'-linked deubiquitination of the transcriptional coactivator WWTR1/TAZ leading to its stabilization and inhibition of osteoclastogenesis (By similarity). Deubiquitinates and stabilizes epidermal growth factor receptor EGFR to prevent its degradation and to activate its downstream mediators (By similarity). Modulates oxidative activity in skeletal muscle by regulating key mitochondrial oxidative proteins (By similarity). Enhances the activity of hypoxia-inducible factor 1-alpha/HIF1A by abrogateing its VHL E3 ligase-mediated ubiquitination and consequently inhibiting its degradation (PubMed:25615526)