2.1.3.3 carbamoyl phosphate + canaline 66fold higher activity with canaline than with ornithine, enzyme may play a role in canavanine synthesis 2.1.3.3 carbamoyl phosphate + L-Orn-Ala-hArg the enzyme produces Cit-Ala-hArg from tripeptide Orn-Ala-hArg and carbamyl phosphate as substrates 2.1.3.3 carbamoyl phosphate + L-ornithine - 2.1.3.3 carbamoyl phosphate + L-ornithine anabolic function 2.1.3.3 carbamoyl phosphate + L-ornithine enzyme belongs to the arginine biosynthesis pathway 2.1.3.3 carbamoyl phosphate + L-ornithine cytoplasmic form has predominantly catabolic function, mitochondrial form has anabolic function 2.1.3.3 carbamoyl phosphate + L-ornithine catalyzes the last step of mitochondrial citrulline synthesis 2.1.3.3 carbamoyl phosphate + L-ornithine first step in arginine biosynthesis 2.1.3.3 carbamoyl phosphate + L-ornithine enzyme is involved in the biosynthesis of arginine in many organisms and participates in the urea cycle of mammals 2.1.3.3 carbamoyl phosphate + L-ornithine enzyme seems to be involved in a bicarbonate-fixing pathway 2.1.3.3 carbamoyl phosphate + L-ornithine ornithine transcarbamylase deficiency is the most common inherited disorder of the urea cycle and is transmitted as an X-linked trait, defects in the OTC gene, especially at clusters of the substrate binding sites, cause a block in ureagenesis resulting in hyperammonemia, which can lead to brain damage and death 2.1.3.3 carbamoyl phosphate + L-ornithine there is no preferential partitioning of carbamoyl phosphate between the arginine and pyrimidine biosynthetic pathways. Channeling must occur during the dynamic association of coupled enzymes pairs 2.1.3.3 carbamoyl phosphate + L-ornithine Sulfolobus solfataricus lacks ornithine acetyltransferase and thus forms N-acetylglutamate exclusively via the energetically less favourable reaction catalysed by N-acetylglutamate synthase, investing 1 mol of acetyl CoA per mol of N-acetyl intermediate synthesized 2.1.3.3 carbamoyl phosphate + L-ornithine catalyzes the sixth step in arginine biosynthesis 2.1.3.3 additional information involved in arginine metabolism 2.1.3.3 additional information key protein involved in the degradation of arginine during malolactic fermentation 2.1.3.3 additional information Lmo0036 is an ornithine carbamoyltransferase and also a putrescine carbamoyltransferase, EC 2.1.3.6, catalysing reversible ornithine and putrescine carbamoyltransfer reactions