| 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) |
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| 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) |
| 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) |
| carbamyl phosphate | <chemical> Phosphate salt of carbamic acid. Chemical name: Carbamic acid, monoanhydride with phosphoric acid (12 Dec 1998) |
| galactose-1-phosphate | A phosphorylated derivative of galactose that is key in galactose metabolism; accumulates in certain types of galactosaemia. (05 Mar 2000) |
| galactose-1-phosphate uridylyltransferase | <enzyme> An enzyme catalyzing the reaction of UTP and alpha-d-galactose 1-phosphate to form UDPgalactose and pyrophosphate, the second and most important step in the metabolism of d-galactose; a deficiency of this enzyme results in an accumulation of galactose, galactose-1-phosphate, and galactitol. (05 Mar 2000) |
| galactose-6-phosphate dehydrogenase | <enzyme> Possibly a ketoaldose, phosphorylated at primary alcoholic group; found in goat liver cytoplasm, requires nad, has high substrate specificity Registry number: EC 1.1.1.- Synonym: hexose-6-phosphate dehydrogenase (26 Jun 1999) |
| galactose-6-phosphate isomerase | <enzyme> Converts galactose 6-phosphate to tagatose 6-phosphate Registry number: EC 5.3.1.- Synonym: galactose 6-phosphate isomerase, gal-6-p isomerase, tagatose-6-phosphate isomerase (26 Jun 1999) |
| pantetheine 4'-phosphate | The prosthetic group of the acyl carrier protein in the fatty acid synthase complex. Synonym: pantetheine 4'-phosphate. (05 Mar 2000) |
| mannose-1-phosphate guanylyltransferase | <enzyme> A transferase that catalyses the transfer of GDP to the mannose of mannose-1-phosphate. Consider also the bifunctional enzyme, phosphomannose isomerase-guanosine diphospho-d-mannose pyrophosphorylase; rfbm has similarity to long-chain, iron-containing alcohol dehydrogenases Registry number: EC 2.7.7.13 Synonym: GDPmannose phosphorylase, GDP mannose pyrophosphorylase, GTP-alpha-d-mannose-1-phosphate guanylyltransferase, GDP-mannose pyrophosphorylase, rfbm gene product, rfbm protein (26 Jun 1999) |
| mannose-6-phosphate | <biochemistry> Mannose derivative formed by phosphorylation in the Golgi complex of certain mannose residues on N glycan chains of lysosomal enzymes. Believed to function as targetting signal that causes entry of these enzymes to the lysosomes. The receptor (215 kD) is enriched in specialised pre lysosomes. (18 Nov 1997) |
| mannose-6-phosphate isomerase | <enzyme> An enzyme that catalyses the reversible isomerization of d-mannose-6-phosphate to form d-fructose-6-phosphate, an important step in glycolysis. Chemical name: D-Mannose-6-phosphate ketol isomerase Registry number: EC 5.3.1.8 (12 Dec 1998) |
| mannose-6-phosphate receptors | Receptors in Golgi apparatus to which newly synthesised proteins that are destined to enter lysosomes bind. (05 Mar 2000) |
| mannose-6-phosphate reductase | <enzyme> Involved in biosynthesis of mannitol in celery Registry number: EC 1.1.1.224 Synonym: manp reductase (26 Jun 1999) |