Genetic Variants of Glucose 6-Phosphate Dehydrogenase from Human Erythrocytes: Unique Properties of the A - Variant Isolated from “Deficient” Cells
- 1 April 1972
- journal article
- Published by Proceedings of the National Academy of Sciences in Proceedings of the National Academy of Sciences
- Vol. 69 (4) , 946-950
- https://doi.org/10.1073/pnas.69.4.946
Abstract
The A(-) type of glucose 6-phosphate dehydrogenase (EC 1.1.1.49) has been isolated from human erythrocytes deficient in this enzyme. The specific activity of the purified protein is similar to that previously reported for the enzyme isolated from normal, nondeficient erythrocytes. During the purification procedure, a portion of the A(-) enzyme converts spontaneously, from the native "fraction I", to a "fraction II" having different kinetic and chromatographic properties. The conversion of fraction I to II can be reproduced freely by treatment with iodosobenzoate, and fraction II can be converted back to fraction I by treatment with dithioglycol. We suggest that fraction II is an enzyme species in which one or more sulfhydryl groups have been oxidized to disulfide(s). The tendency to oxidation appears to be a property specific to the A(-) variant and may represent the basis for its rapid rate of inactivation and consequent deficiency in vivo.Keywords
This publication has 24 references indexed in Scilit:
- Genetic variants of human erythrocyte glucose 6-phosphate dehydrogenase. I. Regulation of activity by oxidized and reduced nicotinamide-adenine dinucleotide phosphateBiochemistry, 1971
- Amino acid substitution (histidine to tyrosine) in a glucose-6-phosphate dehydrogenase variant (G6PD Hektoen) associated with over-productionJournal of Molecular Biology, 1970
- Distinctive patterns of NADP binding to dimeric and tetrameric glucose 6-phosphate dehydrogenase from human red cellsBiochemical and Biophysical Research Communications, 1970
- Enzymic properties of different types of human erythrocyte glucose-6-phosphate dehydrogenase, with characterization of two new genetic variantsJournal of Clinical Investigation, 1968
- Electrophoretic behaviour of human erythrocyte glucose 6-phosphate dehydrogenase during purificationBiochemical and Biophysical Research Communications, 1968
- In vivo lability of glucose-6-phosphate dehydrogenase in GdA- and Gdmediterranean deficiencyJournal of Clinical Investigation, 1968
- Resolution of genetic variants of human erythrocyte glucose 6-phosphate dehydrogenase by thin-layer chromatographyBiochemical and Biophysical Research Communications, 1967
- Negro Variant of Glucose-6-Phosphate Dehydrogenase Deficiency (A - ) in ManScience, 1967
- Different properties of glucose 6-phosphate dehydrogenase from human erythrocytes with normal and abnormal enzyme levelsBiochemical and Biophysical Research Communications, 1965
- ERYTHROCYTE GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY: EVIDENCE OF DIFFERENCES BETWEEN NEGROES AND CAUCASIANS WITH RESPECT TO THIS GENETICALLY DETERMINED TRAIT*Journal of Clinical Investigation, 1959