Computer simulation of the metabolic consequences of the combined deficiency of 6-phosphogluconolactonase and glucose-6-phosphate dehydrogenase in human erythrocytes

Thorburn, D.R.; Kuchel, P.W.

Journal of Laboratory and Clinical Medicine 110(1): 70-74

1987


ISSN/ISBN: 0022-2143
PMID: 3598338
Document Number: 297086
Computer simulation of the human erythrocyte hexose monophosphate shunt was used to investigate whether the severity of hemolysis, caused by deficiency of glucose-6-phosphate dehydrogenase, could be influenced by an accompanying deficiency of 6-phosphogluconolactonase. The known kinetics of the enzymes involved in the oxidative part of the hexose monophosphate shunt suggest that partial deficiency of 6-phosphogluconolactonase would not restrict the rate of reduced nicotinamide-adenine dinucleotide phosphate production significantly. It is therefore concluded that the metabolic consequences of a combined deficiency of the two enzymes are unlikely to be responsible for the interpatient variability of the hematologic response to deficiency of glucose-6-phosphate dehydrogenase.

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