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). OTCD is a disorder of the urea cycle characterized by hyperammonemia. In males, OTCD is fatal, whereas females express variable symptoms. In addition, the OTC gene localizes near a region of the X chromosome that is associated with Duchenne muscular dystrophy, suggesting a possible role in that disease as well.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFIHCELISA
recommended for detection of OTC of mouse, rat, human and canine origin by WB, IP, IF, IHC(P) and ELISA