COMPUTER-SIMULATION OF THE METABOLIC CONSEQUENCES OF THE COMBINED DEFICIENCY OF 6-PHOSPHOGLUCONOLACTONASE AND GLUCOSE-6-PHOSPHATE-DEHYDROGENASE IN HUMAN-ERYTHROCYTES

Abstract

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 variabi..