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Carbamoyl phosphate
- Carbamoyl phosphate synthetase I deficiency is an inherited disorder that causes ammonia to accumulate in the blood (hyperammonemia). Ammonia, which is formed when ...
- ... CPS1 gene provides instructions for making the enzyme carbamoyl phosphate synthetase I. This enzyme participates in the urea ... ammonia, which is toxic.The specific role of carbamoyl phosphate synthetase I is to control the first step ...
- ... acetylglutamate is necessary to turn on the enzyme carbamoyl phosphate synthetase I. This enzyme controls the first step ... at all. This shortage of N-acetylglutamate prevents carbamoyl phosphate synthetase I from being turned on, which prevents ...
- ... to control the reaction in which two compounds, carbamoyl phosphate and ornithine, form a new compound called citrulline. ...
- ... body. These diseases include: Argininosuccinic aciduria Arginase deficiency Carbamyl phosphate synthetase (CPS) deficiency Citrullinemia N-acetylglutamate synthetase (NAGS) ...