carbamoyl phosphate synthetase


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car·ba·mo·yl phos·phate syn·the·tase

a phosphotransferase catalyzing the formation of carbamoyl phosphate. There are two significant isozymes. Carbomoyl phosphate synthetase I is a mitochondrial enzyme that catalyzes the reaction of 2ATP, NH3, CO2, and H2O to carbamoyl phosphate, 2ADP, and orthophosphate. It is activated by N-acetylglutamate and participates in urea biosynthesis. A deficiency of carbamoyl phosphate synthetase I can result in hyperammonemia. 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 participates in pyrimidine biosynthesis.
References in periodicals archive ?
These enzymes are carbamoyl phosphate synthetase 1 (CPS1), ornithine transcarbamylase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase and N-acetylglutamate synthetase.
Hepatocyte paraffin antigen 1 (Hep Par 1), which recognizes the urea cycle enzyme carbamoyl phosphate synthetase, (1) and polyclonal antibody directed against carcinoembryonic antigen (pCEA) are among the most commonly used hepatocellular markers, but they have less than 50% sensitivity for poorly differentiated HCC.
The antigen for Hep Par 1 antibody is the urea cycle enzyme carbamoyl phosphate synthetase 1.
Understanding carbamoyl phosphate synthetase deficiency: impact of clinical mutations on enzyme functionality.
1,2) This antigen was shown to be carbamoyl phosphate synthetase 1, an enzyme involved in urea synthesis.