Ubiquitin Carboxyl-Terminal Hydrolase 14 (USP14) Antibody – Rabbit Polyclonal

This gene encodes a member of the ubiquitin-specific processing (UBP) family of proteases that is a deubiquitinating enzyme (DUB) with His and Cys domains. This protein is located in the cytoplasm and cleaves the ubiquitin moiety from ubiquitin-fused precursors and ubiquitinylated proteins. Mice with a mutation that results in reduced expression of the ortholog of this protein are retarded for growth, develop severe tremors by 2 to 3 weeks of age followed by hindlimb paralysis and death by 6 to 10 weeks of age. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008]

USP14: Proteasome-associated deubiquitinase which releases ubiquitin from the proteasome targeted ubiquitinated proteins. Ensures the regeneration of ubiquitin at the proteasome. Is a reversibly associated subunit of the proteasome and a large fraction of proteasome-free protein exists within the cell. Required for the degradation of the chemokine receptor CXCR4 which is critical for CXCL12-induced cell chemotaxis. Serves also as a physiological inhibitor of endoplasmic reticulum-associated degradation (ERAD) under the non-stressed condition by inhibiting the degradation of unfolded endoplasmic reticulum proteins via interaction with ERN1. Indispensable for synaptic development and function at neuromuscular junctions (NMJs). Homodimer (Potential). Associates with the 26S proteasome. Interacts with FANCC, CXCR4 and ERN1. Belongs to the peptidase C19 family. USP14/UBP6 subfamily.

Protein type: Ubiquitin-specific protease; EC 3.4.19.12; Protease; Ubiquitin conjugating system

Chromosomal Location of Human Ortholog: 18p11.32

Cellular Component: cell surface; cytoplasm; cytoplasmic membrane-bound vesicle; plasma membrane; proteasome complex; synapse

Molecular Function: cysteine-type endopeptidase activity; endopeptidase inhibitor activity; protein binding; tRNA guanylyltransferase activity; ubiquitin-specific protease activity

Biological Process: protein deubiquitination; regulation of chemotaxis; synaptic transmission; ubiquitin-dependent protein catabolic process