Schilirò G, Russo A, Mauro L, Pizzarelli G, Marino S
Pediatr Res. 1976 Aug;10(8):739-42. doi: 10.1203/00006450-197608000-00009.
Nine Sicilian children known to be deficient in glucose-6-phosphate dehyrdrogenase (G6PD) were studied to see if there were anomalies of bactericidal activity in peripheral blood phagocytes. The type of deficiency was established. The G6PD levels in the leukocyte were found to be 26% of the controls (0.094 +/- 0.03, normal controls 0.360 +/- 0.12). The Michaelis constant for NADP and glucose-6-phosphate (G6P) was lower than the control. Conversely, the utilization of the analogous 2-deoxyglucose-6-phosphate (2dG6P) and galactose-6-phosphate (Ga16P) was higher. The thermostability of the enzyme in the deficient subjects was lower and the pH optima (8 and 9.5) were different from the controls. An identical electrophoretic pattern was found in both normal and deficient subjects. The bactericidal activity in the deficient subjects was normal. There was no difference in the results of nitroblue tetrazolium (NBT) tests in either group.
对9名已知葡萄糖-6-磷酸脱氢酶（G6PD）缺乏的西西里儿童进行了研究，以观察外周血吞噬细胞的杀菌活性是否存在异常。确定了缺乏的类型。发现白细胞中的G6PD水平为对照组的26%（0.094±0.03，正常对照组为0.360±0.12）。NADP和葡萄糖-6-磷酸（G6P）的米氏常数低于对照组。相反，类似的2-脱氧葡萄糖-6-磷酸（2dG6P）和半乳糖-6-磷酸（Ga16P）的利用率更高。缺乏症患者中该酶的热稳定性较低，最适pH值（8和9.5）与对照组不同。在正常和缺乏症患者中发现了相同的电泳图谱。缺乏症患者的杀菌活性正常。两组的硝基蓝四唑（NBT）试验结果没有差异。
