Ornithine transcarbamylase (OTC) is mapped to human chromosome Xp11.4 It is expressed in hepatocytes and enterocytes and belongs to the transcarbamylase family. Its precursor possesses a N-terminal signal peptide. OTC exists as anabolic and catabolic forms. Structurally, OTC comprises two lobes with the substrate carbamoyl phosphate (CP) binding pocket in between them. The conserved Ser-Thr-Arg-Thr-Arg motif is essential for CP binding.
Synonyms: Anti-OTCase; Anti-Ornithine carbamoyltransferase, mitochondrial precursor; Anti-Ornithine transcarbamylase
Storage: -20C
Application: Anti-OTC antibody produced in rabbit has been used in:
•western blotting
•immunofluorescence
•immunohistochemical staining
Biochem Physiol Actions: Ornithine transcarbamylase (OTC), a urea cycle enzyme, catalyzes the conversion carbamoyl phosphate and ornithine to citrulline. It is a key enzyme for ammonia synthesis as well as for the catabolism of amino acids. The active site lysine 88 residue is crucial for binding carbamoyl phosphate. The anabolic OTC is involved in the urea cycle and arginine biosynthesis. The catabolic OTC participates in the arginine deiminase pathway and citrulline breakdown. A deficiency in OTC leads to urea cycle disruption resulting in ornithine transcarbamylase disorder (OTCD).
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