9001-69-8

Basic information

• Product Name: CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE
• Synonyms: EC 2.1.3.3;CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE;ORNITHINE TRANSCARBAMYLASE;ORNITHINE CARBAMYLTRANSFERASE;ornithine carbamoyl transferase from strept. faec.;ornithine carbamyltransferase from*streptococcus;Carbamoyltransferase, ornithine;ORNITHINE CARBAMYLTRANSFERASE FROM*STREP TOCOCCUS FA
• CAS NO: 9001-69-8
• Molecular Weight: 0
• EINECS: 232-625-1
• Mol File: 9001-69-8.mol
• Article Data: 0

Chemical Properties

• Appearance: /lyophilized powder
• Storage Temp.: −20°C
• CAS DataBase Reference: CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE(CAS DataBase Reference)
• NIST Chemistry Reference: CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE(9001-69-8)
• EPA Substance Registry System: CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE(9001-69-8)

Safety Data

• WGK Germany: 3
• Hazardous Substances Data: 9001-69-8(Hazardous Substances Data)

Usage

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.