Identification | Back Directory | [Name]
CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE | [CAS]
9001-69-8 | [Synonyms]
OTCase EC 2.1.3.3 ORNITHINE TRANSCARBAMYLASE ORNITHINE CARBAMYLTRANSFERASE Carbamoyltransferase, ornithine ornithine carbamyltransferase from*streptococcus ornithine carbamoyl transferase from strept. faec. CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE ORNITHINE CARBAMYLTRANSFERASE FROM*STREP TOCOCCUS FA Native Streptococcus faecalis Ornithine Transcarbamylase ORNITHINE CARBAMOYL TRANSFERASE FROM STREPT. FAEC., 30 U* Ornithine Transcarbamylase from Streptococcus faecalis Carbamoylphosphate:L-Ornithine Carbamoyltransferase, Ornithine Carbamoyl Transferase, OTCase | [EINECS(EC#)]
232-625-1 | [MDL Number]
MFCD00131769 |
Hazard Information | Back Directory | [Description]
An enzyme that catalyzes the transfer of a carbamyl
group from carbamyl phosphate to ornithine to form citrulline; it is a constituent reaction of the urea cycle. This enzyme normally
occurs in the mitochondria of liver cells, but only to a minute extent
in serum. Since it does not occur to a significant extent in serum,
kidney, brain, etc., and only 1% or 2% of the hepatic concentration
occurs in small intestine, its presence in serum is a specific indica_x0002_tion of damage to liver cells. | [Biochem/physiol Actions]
Mutations in ornithine transcarbamylase cause Ornithine transcarbamylase deficiency which prevents the clearance of the byproduct ammonia leading to hyperammonemia. |
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