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.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
General description
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.
Immunogen
Synthetic peptide directed towards the N terminal region of human OTC
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Sequence
Synthetic peptide located within the following region: AFRNGHNFMVRNFRCGQPLQNKVQLKGRDLLTLKNFTGEEIKYMLWLSAD
Specificity
Anti-OTC polyclonal antibody reacts with canine, human, mouse, rat, bovine, and pig ornithine carbamoyltransferase/ornithine transcarbamylase(s).
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