See Also: carbamoyl phosphate synthetase(medicine)
carbamoyl phosphate(medicine)
carbamoyl-phosphate synthase (ammonia)(medicine)
carbamoyl-phosphate synthase (glutamine-hydrolyzing)(medicine)
hexose phosphate synthetase(medicine)
bis(monoacylglycero)phosphate synthetase(medicine)
N-(5-amino-1-ribosyl-4-imidazolylcarbonyl)-L-aspartic acid 5'-phosphate synthetase(medicine)
Herbal medicine (botanical medicine, herbology, phytomedicine)(health)
Carbamoyl(medicine)
UDP-GlcNAc-undecaprenyl phosphate N-acetylglucosaminyl 1-phosphate transferase(medicine)

carbamoyl phosphate synthetase (medicine)


carbamoyl phosphate synthetase


A phosphotransferase catalyzing the formation of carbamoyl phosphate. There are two significant isozymes. Carbomoyl phosphate synthetase I is a mitochondrial enzyme that catalyses the reaction of 2ATP, NH3, CO2, and H2O to carbamoyl phosphate, 2ADP, and Pi. It is activated by N-acetylglutamate and participates in urea biosynthesis.

A deficiency of carbamoyl phosphate synthetase I can result in hyperammonaemia. Carbamoyl phosphate synthetase II is a cytosolic enzyme that, under physiological conditions, uses l-glutamine as the nitrogen source (producing l-glutamate) instead of NH3, is not activated by N-acetylglutamate, and is found in pyrimidine biosynthesis.