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glucose-1-phosphate kinase <enzyme> An enzyme that, in the presence of ATP, catalyses the phosphorylation of d-glucose 1-phosphate to form d-glucose 1,6-bisphosphate and ADP; found in yeast and muscle; d-glucose 1,6-bisphosphate is a required cofactor of one of the enzymes in glycogenolysis.
Synonym: glucose-1-phosphate kinase.
(05 Mar 2000)
glucose-1-phosphate phosphodismutase A phosphotransferase catalyzing the reversible transfer of a phosphate residue from one d-glucose 1-phosphate to another, yielding d-glucose 1,6-bisphosphate and d-glucose. This enzyme provides a crucial intermediate needed for glucose-phosphate isomerase.
(05 Mar 2000)
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)
glucose-1-phospho-D-mannosylglycoprotein phosphodiesterase <enzyme> Removes the glucose-1-phosphate from glc-alpha-1-p-6-mannose residues in glycoproteins as a unit; pH optimum 7.5
Registry number: EC 3.1.4.51
Synonym: ag1p phosphodiesterase, alpha-glucose-1-phosphate phosphodiesterase
(26 Jun 1999)
glucose-3-phosphatase <enzyme> From rat liver; has glucose-3-phosphate hydrolytic activity
Registry number: EC 3.1.3.-
(26 Jun 1999)
glucose-6-dehydrogenase deficiency <biochemistry> An inherited condition that results in a deficiency in glucose-6-phosphate dehydrogenase. Particular drugs (sulphonamides) can exacerbate this problem. The result is haemolytic anaemia.
(27 Sep 1997)
glucose-6-phosphatase <enzyme> An enzyme that catalyses the conversion of d-glucose-6-phosphate and water to d-glucose and orthophosphate. This enzyme is deficient in glycogen storage disease Ia.
Chemical name: D-Glucose-6-phosphate phosphohydrolase
Registry number: EC 3.1.3.9
(12 Dec 1998)
glucose-6-phosphatase hepatorenal glycogenosis Glycogenosis due to glucose-6-phosphatase deficiency, resulting in accumulation of excessive amounts of glycogen of normal chemical structure, particularly in liver and kidney.
Synonym: Gierke's disease, glucose-6-phosphatase hepatorenal glycogenosis, von Gierke's disease.
(05 Mar 2000)
glucose-6-phosphate <biochemistry> Glucose 6-phosphate is a phosphomonoester of glucose that is formed by transfer of phosphate from ATP, catalysed by the enzyme hexokinase.
It is an intermediate both of the glycolytic pathway (next converted to fructose 6 phosphate) and of the NADPH generating pentose phosphate pathway, formed from glucose via hexokinase. However it is not strictly a glycolytic intermediate and it is readily converted to glycogen or oxidized to NADPH.
(10 Oct 1997)
glucose-6-phosphate dehydrogenase <enzyme> An NADP+ enzyme that catalyses the dehydrogenation (oxidation) of d-glucose-6-phosphate to 6-phospho-d-glucono-d-lactone, this reaction initiating the Dickens shunt.
Deficiency of this enzyme is the commonest disease-causing enzyme defect in humans affecting an estimated 400 million people.
The gene for this enzyme is on the X chromosome. Males with the enzyme deficiency develop haemolytic anaemia when red blood cells are exposed to oxidant drugs such as the antimalarial primaquine, the sulfonamide antibiotics or sulfones, naphthalene moth balls, or fava beans.
Synonym: Robison ester dehydrogenase, Zwischenferment.
Acronym: G6PD
(12 Sep 2002)
glucose-6-phosphate dehydrogenase deficiency A deficiency of glucose-6-phosphate dehydrogenase, an enzyme important for maintaining cellular concentrations of reduced nucleotides.
Deficiency of this enzyme is the commonest disease-causing enzyme defect in humans affecting an estimated 400 million people.
The gene for this enzyme is on the X chromosome and there are various polymorphic forms.
Males with the enzyme deficiency develop haemolytic anaemia when red blood cells are exposed to oxidant drugs such as the antimalarial primaquine, the sulfonamide antibiotics or sulfones, naphthalene moth balls, or fava beans. It can also cause anaemia of the newborn, and chronic nonspherocytic haemolytic anaemia.
Inheritance: X-linked.
(12 Sep 2002)
glucose-6-phosphate isomerase <enzyme> An enzyme that catalyses the reversible interconversion of glucose-6-phosphate and fructose-6-phosphate, and is a part of the glycolytic and gluconeogenic pathways. Deficiency of the enzyme, an autosomal recessive trait, results in liver glycogenesis and haemolytic anaemia.
Chemical name: D-Glucose-6-phosphate ketol-isomerase
Registry number: EC 5.3.1.9
(12 Dec 1998)
glucose-6-phosphate translocase <enzyme> A component of EC 3.1.3.9 which transports glucose phosphate into endoplasmic reticulum
Registry number: EC 2.7.-
Synonym: t1 transport protein
(26 Jun 1999)
glucose clamp technique <technique> Maintenance of a constant blood glucose level by perfusion or infusion with glucose or insulin. It is used for the study of metabolic rates (e.g., in glucose, lipid, amino acid metabolism) at constant glucose concentration.
(12 Dec 1998)
glucose dehydrogenase <enzyme> Converts beta-d-glucose to d-glucono-d-lactone, transferring hydrogen to NAD+ or NADP+.
Compare: glucose oxidase.
(05 Mar 2000)
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