Thorburn D R, Kuchel P W
J Lab Clin Med. 1987 Jul;110(1):70-4.
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.
利用人体红细胞磷酸己糖旁路的计算机模拟来研究，葡萄糖-6-磷酸脱氢酶缺乏所致溶血的严重程度是否会受到伴发的6-磷酸葡糖酸内酯酶缺乏的影响。磷酸己糖旁路氧化部分所涉及酶的已知动力学表明，6-磷酸葡糖酸内酯酶的部分缺乏不会显著限制还原型烟酰胺腺嘌呤二核苷酸磷酸的生成速率。因此得出结论，这两种酶联合缺乏的代谢后果不太可能是葡萄糖-6-磷酸脱氢酶缺乏患者血液学反应个体差异的原因。
