carbamoyl-phosphate synthase (ammonia)
- carbamoyl-phosphate synthase (ammonia)
- car·bam·o·yl-phos·phate syn·thase (am·mo·nia) (kahr-bamґo-əl-fosґfāt sinґthās ə-moґne-ə) [EC 6.3.4.16] an enzyme of the ligase class that catalyzes the synthesis of carbamoyl phosphate from ammonia and carbon dioxide, the first committed step in the urea cycle (see illustration at urea cycle, under cycle). The reaction occurs predominantly in liver mitochondria, requires N-acetylglutamate as a cofactor, and hydrolyzes two molecules of ATP. Decreased enzyme activity, an autosomal recessive trait, causes carbamoyl phosphate synthetase deficiency.
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2011.
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carbamoyl phosphate synthetase — (CPS) car·bam·o·yl phos·phate syn·the·tase (CPS) (kahr bamґo əl fosґfāt sinґthə tās) 1. carbamoyl phosphate synthase (ammonia); called also carbamoyl phosphate synthetase I (CPSI). 2. carbamoyl phosphate synthase (glutamine… … Medical dictionary
carbamoyl phosphate synthetase deficiency — car·bam·o·yl phos·phate syn·the·tase de·fi·cien·cy (kahr bamґo əl fosґfāt sinґthə tās) an autosomal recessive aminoacidopathy caused by mutations in the CPS1 gene (locus: 2q35), which encodes carbamoyl phosphate synthase… … Medical dictionary
CTP synthase — Cytidine triphosphate Identifiers EC number 6.3.4.2 … Wikipedia
N-Acetylglutamate synthase deficiency — Classification and external resources N Acetylglutamic acid OMIM 237310 … Wikipedia
CPSI — carbamoyl phosphate synthetase I; see carbamoyl phosphate synthase (ammonia) … Medical dictionary
List of EC numbers (EC 6) — This list contains a list of EC numbers for the sixth group, EC 6, ligases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 6.1: Forming Carbon Oxygen… … Wikipedia
urea cycle — a series of metabolic reactions, occurring in the liver, by which ammonia is converted to urea using cyclically regenerated ornithine as a carrier. See illustration. Urea cycle. Diagrammatic representation of reactions by which excess nitrogen in … Medical dictionary
N-acetylglutamic acid — N ac·e·tyl·glu·tam·ic ac·id (as″ə təl ) (as″ə tēl″gloo tamґik) glutamic acid acetylated at its N terminus, a necessary cofactor in the synthesis of carbamoyl phosphate catalyzed by carbamoyl phosphate… … Medical dictionary
Metabolism — Cell metabolism redirects here. For the journal, see Cell Metabolism. Structure of adenosine triphosphate, a central intermediate in energy metabolism Metabolism (from Greek: μεταβολή metabolē , change or Greek: μεταβολισμός metabolismos,… … Wikipedia
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