Mitochondrial ornithine transcarbamylase (OTC) trimer catalyzes the reaction of carbamoyl phosphate and ornithine to form citrulline and orthophosphate. While the enzyme has been purified and characterized (Tamir and Ratner 1963a,b; Tsuji 1983), and the gene encoding it has been cloned (Shimogiri et al. 1998), the physiological role of this reaction is unclear. Indirect studies in vivo indicate that chickens cannot synthesize citrulline from ornithine and the chicken enzyme is undetectable in liver, of low abundance in other tissues such as kidney, and of low specific activity compared to homologous proteins purified in parallel from mammalian sources.
Tsuji, S, Mizutani, M, Shimogiri, T, Mannen, H, Kono, M
Ratner, S, Tamir, H
Tsuji, S
ornithine carbamoyltransferase activity of OTC trimer [mitochondrial matrix]
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