ornithine carbamoyltransferase

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Related to ornithine carbamoyltransferase: ornithine carbamoyltransferase deficiency


an amino acid obtained from arginine by splitting of urea; it is an intermediate in urea biosynthesis.
ornithine carbamoyltransferase (ornithine transcarbamylase) an enzyme that catalyzes a reaction occurring in the liver mitochondria as part of the urea cycle.

or·ni·thine car·bam·o·yl·trans·fer·ase

an enzyme catalyzing formation of l-citrulline and orthophosphate from l-ornithine and carbamoyl phosphate; a part of the urea cycle; a deficiency of this enzyme will result in ammonia intoxication and impaired urea formation.

ornithine carbamoyltransferase

/or·ni·thine car·ba·mo·yl·trans·fer·ase/ (kahr″bah-mo″il-trans´fer-ās) an enzyme that catalyzes the carbamoylation of ornithine to form citrulline, a step in the urea cycle; deficiency of the enzyme is an X-linked aminoacidopathy causing hyperammonemia, neurologic abnormalities, and oroticaciduria and is usually fatal in the neonatal period in males.

ornithine carbamoyltransferase

an enzyme in the blood that increases in patients with liver and other diseases. Its normal concentration in serum is 8 to 20 mIU/mL. Also called ornithine transcarbamoylase.
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