CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE Chemical Properties

storage temp. 

−20°C

form 

lyophilized powder

Safety Information

WGK Germany 

3

MSDS

• Language:English Provider:SigmaAldrich

CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE Usage And Synthesis

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.

CARBAMOYLPHOSPHATE: L-ORNITHINE CARBAMOYLTRANSFERASE(9001-69-8)Related Product Information

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• Cupric acetylacetonate
• N-BUTYLISOCYANIDE
• Tris(2,4-pentanedionato)chroMiuM(III)
• METHYL ISOCYANOACETATE
• Ethyl isocyanoacetate
• TERT-BUTYL ISOCYANIDE
• SALCOMINE
• Benzyl isocyanide
• TRIS(2,2,6,6-TETRAMETHYL-3,5-HEPTANEDIONATO)DYSPROSIUM(III)
• Ferric acetylacetonate
• DICHLORO(ETHYLENEDIAMINE)PLATINUM(II)
• 2,4-PENTANEDIONE, SILVER DERIVATIVE
• COBALT(II) ACETYLACETONATE
• TRIS(2,2,6,6-TETRAMETHYL-3,5-HEPTANEDIONATO)EUROPIUM(III)
• PHENYLSELENOL
• Aluminum acetylacetonate
• 1,1,3,3-TETRAMETHYLBUTYL ISOCYANIDE