OTC polyclonal antibody

OTC (ornithine carbamoyltransferase), also known as OTCase, is a 354 amino acid protein that belongs to the ATCase/OTCase family of proteins. Expressed in liver and intestinal mucosa, OTC localizes to the mitochondrial matrix and exists as a homotrimer. Specifically, OTC plays a vital role in the urea cycle, catalyzing the second step in this pathway: the formation of L-citrulline from L-orthinine and carbamoyl phosphate. In humans, the urea cycle is an important pathway to detoxification of ammonia. Mutations in the gene encoding OTC are associated with the X-linked disorder OTCD (ornithine carbamoyltransferase deficiency).