UBA1 Antibody – Polyclonal

The protein encoded by this gene catalyzes the first step in ubiquitin conjugation to mark cellular proteins for degradation. This gene complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair. It is part of a gene cluster on chromosome Xp11.23. Alternatively spliced transcript variants that encode the same protein have been described. [provided by RefSeq, Jul 2008]

UBE1: an enzyme of the ubiquitin-activating E1 family. Adenylates ubiquitin at its carboxy-terminal glycine residue and thereafter links this residue to the side chain of a cysteine residue in E1, yielding an ubiquitin-E1 thiolester. There are two active sites within the E1 molecule, allowing it to accomodate two ubiquitin moieties at a time, with a new ubiquitin forming an adenylate intermediate as the previous one is transferred to the thiol site. Complements an X-linked mouse temperature-sensitive defect in DNA synthesis, and thus may function in DNA repair.

Protein type: Ubiquitin conjugating system; Ubiquitin ligase; EC 6.3.2.19

Chromosomal Location of Human Ortholog: Xp11.23

Cellular Component: cytoplasm; cytosol; desmosome; endosome membrane; heterochromatin; lysosomal membrane; mitochondrion; nucleus; rough endoplasmic reticulum membrane

Molecular Function: ATP binding; protein binding; ubiquitin activating enzyme activity; ubiquitin-protein ligase activity

Biological Process: modification-dependent protein catabolic process; protein ubiquitination; response to DNA damage stimulus

Disease: Spinal Muscular Atrophy, X-linked 2