| 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) |
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| 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) |