Ornithine carbamoyltransferase/ornithine transcarbamylase is a mitochondrial enzyme that maintains the urea cycle by regenerating citrulline (Cit) from carbamoyl phosphate and ornithine; wherein ornithine is generated during the catabolism of arginine to release urea for excretion.
Synonyms: Anti-OCTD; Anti-Ornithine carbamoyltransferase
Storage: -20C
Application: Anti-OTC polyclonal antibody is used to tag ornithine transcarbamylase for detection and quantitation by Western blotting and in plasma by immunohistochemical (IHC) techniques. It is used as a probe to determine the roles of ornithine transcarbamylase in the urea cycle.
Biochem Physiol Actions: OTC is a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also.This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also.