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PMID:1953691

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Citation

Neifer, S, Jung, A and Bienzle, U (1991) Characterization of erythrocytic glucose-6-phosphate dehydrogenase in a mouse strain with reduced G6PD activity. Biomed. Biochim. Acta 50:233-8

Abstract

Erythrocytic glucose-6-phosphate dehydrogenase (G6PD) of a mutant mouse strain with X-linked G6PD-deficiency was purified and compared with the wildtype G6PD by biochemical and physiological characteristics. The red cell G6PD activity of the mutant was 20% of the wildtype. The Michaelis constant (Km) of the substrate glucose-6-phosphate (G6P) was higher (90 microM) for the mutant than for the wildtype (56 microM). The isoelectric focusing with a pH range from 3 to 10 showed one more enzymatically active band for the wildtype G6PD compared to the mutant enzyme. Other enzyme characteristics, however, such as Km for nicotinamide adenine dinucleotide phosphate (NADP), utilisation of 2-deoxy-glucose-6-phosphate (2dG6P) and deamino-NADP, heat stability, pH optimum, molecular weight, and glucose metabolisation via the pentosephosphate pathway were similar in mutant and wildtype enzyme.

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Keywords

Animals; Erythrocytes/enzymology; Glucose-6-Phosphate; Glucosephosphate Dehydrogenase/blood; Glucosephosphate Dehydrogenase/chemistry; Glucosephosphate Dehydrogenase/genetics; Glucosephosphate Dehydrogenase/metabolism; Glucosephosphate Dehydrogenase Deficiency/enzymology; Glucosephosphate Dehydrogenase Deficiency/genetics; Glucosephosphates/metabolism; Hydrogen-Ion Concentration; Isoelectric Focusing; Male; Mice; Mice, Inbred C3H; Mice, Mutant Strains; Molecular Weight; Mutation; NADP/metabolism

Significance

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Gene product Qualifier GO ID GO term name Evidence Code with/from Aspect Notes Status


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