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,
ornithine [or´nĭ-thēn]
ornithine (ôr´n
thēn´),
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.
ornithine [or´nĭ-thēn]
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.
ornithine (ôr´n
thēn´),n an amino acid, not a constituent of proteins, that is produced as an important intermediate substance in the urea cycle.
ornithine carbamoyltransferase,
n an enzyme in the blood that increases in patients with liver and other diseases.
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