| CP | Carbamoyl phosphate |
|---|---|
| CPS | Carbamoyl phosphate synthetase |
| CPSase | Carbamoyl phosphate synthetase |
| OCTase | Ornithine carbamoyl transferase |
| Oct | Ornithine carbamoyl transferase |
| carbamoyl | The acyl radical, NH2-CO-, the transfer of which plays an important role in certain biochemical reactions; e.g., in the urea cycle, via carbamoyl phosphate. (05 Mar 2000) |
|---|---|
| carbamoyl phosphate | H2NCO-OPO32-;a reactive intermediate capable of transferring its carbamoyl group (H2NCO-) to an acceptor molecule, forming citrulline from ornithine in the urea cycle, and ureidosuccinic acid from aspartic acid in pyrimidine ring formation. (05 Mar 2000) |
| 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. (05 Mar 2000) |
| carbamoyl-phosphate synthase (ammonia) | <enzyme> An enzyme that catalyses the formation of carbamoyl phosphate from ATP, carbon dioxide, and ammonia. This enzyme is specific for arginine biosynthesis or the urea cycle. Chemical name: Carbon dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating) Registry number: EC 6.3.4.16 (12 Dec 1998) |
| carbamoyl-phosphate synthase (glutamine-hydrolyzing) | <enzyme> An enzyme that catalyses the formation of carbamoyl phosphate from ATP, carbon dioxide, and glutamine. This enzyme is important in the de novo biosynthesis of pyrimidines. Chemical name: Carbon dioxide:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating) Registry number: EC 6.3.5.5 (12 Dec 1998) |
| carbamoylaspartate dehydrase | dihydro-orotase |
| carbamoylation | Transfer of the carbamoyl from a carbamoyl-containing molecule (e.g., carbamoyl phosphate) to an acceptor moiety such as an amino group. (05 Mar 2000) |
| carbamoylcarbamic acid | NH2CONHCOOH; urea carbonic acid;its amide is biuret (allophanamide). Synonym: carbamoylcarbamic acid, N-carboxyurea. (05 Mar 2000) |
| carbamoylphosphate synthetase deficiency | <biochemistry> Carbamoylphosphate synthetase is the initial enzyme of the urea cycle, catalysing the synthesis of carbamoylphosphate from ammonia, bicarbonate and ATP as the first step of ammonia detoxification. The enzyme is an intramitochondrial form called CPS I. A different isozyme found in the cytoplasm, called CPS II, is much less active and apparently not involved in the urea cycle. The deficiency state is autosomal recessive and presents in infancy with massive hyperammonaemia and neurologic deficits in survivors. Diagnosis is suggested by the blood biochemistry and confirmed by specific enzyme assay on liver or rectal biopsy. Prenatal diagnosis by molecular methods has been used successfully in informative families. Inheritance: autosomal recessive. (07 Apr 1998) |
| carbamoyltransferase | Enzymes transferring carbamoyl groups from one compound to another (e.g., aspartate carbamoyltransferase, ornithine carbamoyltransferase). Synonym: transcarbamoylase. (05 Mar 2000) |
| carbamoylurea | <chemistry> A white, crystalline, nitrogenous substance, C2O2N3H5, formed by heating urea. It is intermediate between urea and cyanuric acid. Origin: Pref. Bi- + urea. Source: Websters Dictionary (01 Mar 1998) |
| carboxyl and carbamoyl transferases | <enzyme> A group of enzymes that catalyze the transfer of carboxyl- or carbamoyl- groups. Registry number: EC 2.1.3 (12 Dec 1998) |
|---|---|
| N-carbamoyl-D-amino acid amidohydrolase | <enzyme> From comamonas sp.e222c; mw 120 kD; hydrolyzes d-enantiomers of various n-carbamoyl-d-amino acids to d-amino acids, ammonia and co2; sensitive to thiol reagents; does not require metal ions Registry number: EC 3.5.1.- Synonym: caa-amidohydrolase, carbamoyl d-aa amidohyrolase (26 Jun 1999) |
| 1-carbamoyl-L-amino acid amidohydrolase | <enzyme> From bacillus stearothermophilus; amino acid sequence given in first source Registry number: EC 3.5.1.- Synonym: carbamoyl l-aa amidohydrolase, n-carbamyl-l-amino acid amidohydrolase (26 Jun 1999) |
Synonyms : CP Synthase I, Carbamoyl-Phosphate Synthetase (Ammonia), Carbamoyl-Phosphate Synthetase I, Carbamoylphosphate Synthetase I, Carbamyl Phosphate Synthase (Ammonia), Carbamyl-Phosphate Synthase (Ammonia), I, Carbamoyl-Phosphate Synthetase, Synthase I, CP
Synonyms : Carbamoyl-Phosphate Synthase (Glutamine), Carbamoylphosphate Synthetase II, Carbamyl Phosphate Synthase II, Carbamyl-Phosphate Synthase (Glutamine), Synthetase II, Carbamoylphosphate
Synonyms : Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease, Carbamoyl-Phosphate Synthetase I Deficiency Disease, Carbamoylphosphate Synthetase I Deficiency Disease, Carbamyl Phosphate Synthetase Deficiency Disease
| carbamoyl |
the radical NH2CO—. Called also carbamyl.
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| carbamoyl phosphate synthetase |
1. carbamoyl-phosphate synthase (ammonia); called also carbamoyl phosphate synthetase I (CPSI). 2. carbamoyl-phosphate synthase (glutamine-hydrolyzing); called also carbamoyl phosphate synthetase II (CPSII).
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| carbamoyl phosphate synthetase deficiency |
a genetic aminoacidopathy due to a deficiency of carbamoyl phosphate synthase (ammonia); characteristic symptoms include pronounced hyperammonemia without oroticaciduria, protein intolerance, and neurologic disorders. Symptoms may begin in the neonatal period or appear later in infancy, with varying degrees of severity. Written also carbamoyl phosphate synthetase I (CPSI) deficiency.
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| carbamoyl-phosphate synthase (ammonia) |
[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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| carbamoyl-phosphate synthase (glutamine-hydrolyzing) |
[EC 6.3.5.5] an enzyme activity of the trifunctional CAD protein (q.v.); it is a ligase that catalyzes the formation of carbamoyl phosphate as the first step in the biosynthesis of pyrimidine nucleotides. Glutamine is the nitrogen donor in the reaction, which is cytosolic and is inhibited by UTP.
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