| 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) |
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| glucose-1-phosphate uridylyltransferase | <enzyme> An enzyme that activates d-glucose by reacting d-glucose 1-phosphate with UTP, producing pyrophosphate and UDP glucose; a crucial step in glycogen biosynthesis. (05 Mar 2000) |
| hexose-1-phosphate uridylyltransferase | <enzyme> An enzyme that catalyses the transfer of ump from udpglucose to galactose 1-phosphate, forming udpgalactose and glucose 1-phosphate. Deficiency in this enzyme is the major cause of galactosaemia. Chemical name: UDPglucose:alpha-D-galactose-1-phosphate uridylyltransferase Registry number: EC 2.7.7.12 (12 Dec 1998) |
| udpglucose-hexose-1-phosphate uridylyltransferase | <enzyme> An enzyme that catalyses the transfer of ump from udpglucose to galactose 1-phosphate, forming udpgalactose and glucose 1-phosphate. Deficiency in this enzyme is the major cause of galactosaemia. Chemical name: UDPglucose:alpha-D-galactose-1-phosphate uridylyltransferase Registry number: EC 2.7.7.12 (12 Dec 1998) |
| uridylyl removing-uridylyltransferase | <enzyme> Modifies a regulatory protein, pii, which modulates an adenyltransferase which regulates E coli glutamine synthetase Registry number: EC 2.7.7.- Synonym: ur-ut (26 Jun 1999) |
| utp-glucose-1-phosphate uridylyltransferase | <enzyme> An enzyme that catalyses the formation of udpglucose from utp plus glucose 1-phosphate. Chemical name: UTP:alpha-D-glucose-1-phosphate uridylyltransferase Registry number: EC 2.7.7.9 (12 Dec 1998) |
| utp-hexose-1-phosphate uridylyltransferase | <enzyme> An enzyme that catalyses the synthesis of udpgalactose from utp and galactose-1-phosphate. It is present in low levels in foetal and infant liver, but increases with age, thereby enabling galactosaemic infants who survive to develop the capacity to metabolise galactose. Chemical name: UTP:alpha-D-hexose-1-phosphate uridylyltransferase Registry number: EC 2.7.7.10 (12 Dec 1998) |
| UTP-RNA uridylyltransferase | <enzyme> Catalyses addition of single ump residue from utp to 3'-end of RNA primer Registry number: EC 2.7.7.- Synonym: terminal uridylyltransferase, ump transferase (26 Jun 1999) |
| galactose | <biochemistry> Hexose identical to glucose except that orientation of H and OH on carbon 4 are exchanged. A component of cerebrosides and gangliosides, glycoproteins. Lactose, the disaccharide of milk, consists of galactose joined to glucose by a _(1-4) glycosidic link. (18 Nov 1997) |
| galactose-1-phosphatase | <enzyme> From rat brain; requires mg for activity; phosphate is potent inhibitor Registry number: EC 3.1.3.- (26 Jun 1999) |
| galactose-1-phosphate | A phosphorylated derivative of galactose that is key in galactose metabolism; accumulates in certain types of galactosaemia. (05 Mar 2000) |
| galactose 3-O-sulfotransferase | <enzyme> Catalyses transfer of sulfate from adenosine 3'-phosphosulfate to methyl galactosides or terminal n-acetyllactosamine-containing carbohydrate chains Registry number: EC 2.8.2.- (26 Jun 1999) |
| 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) |
| galactose-6-sulfatase | <enzyme> An enzyme that eliminates sulfur from the galactose-6-sulfate residues of certain mucopolysaccharides, producing 3,6-anhydrogalactose residues; it is absent in Morquio's syndrome type A. Synonym: galactose-6-sulfurase. (05 Mar 2000) |