Batishchev A I, Cherniak N B, Tokarev Iu N
Biull Eksp Biol Med. 1977 Dec;84(12):728-31.
Kinetic and electrophoretic properties of 230--300 fold purified preparations of glucose-6-phosphate dehydrogenase (G6PD) from red cells of donors and patients with acute drug hemolytic anemia due to G6PD deficiency were studied. A new abnormal variant of G6PD isolated from red cell of a patient with acute drug hemolytic anemia, which was not described in literature, has been discovered. The abnormal enzyme differs from the normal by decreased Michaelis constant for glucose-6-phosphate and nicotinamide adenine dinucleotide phosphate (NADP), by increased utilization of analogues of substrates--2-deoxy-glucose-6-phosphate and particularly deamino-NADP, by low thermal stability, by the character of pH-dependence, by the appearance of a single band of G6PD activity in polyacrylamide gel electrophoresis.
对来自因葡萄糖-6-磷酸脱氢酶(G6PD)缺乏导致急性药物溶血性贫血的供血者和患者红细胞中230 - 300倍纯化的G6PD制剂的动力学和电泳性质进行了研究。从一名急性药物溶血性贫血患者的红细胞中分离出一种文献中未描述的新型G6PD异常变体。该异常酶与正常酶的不同之处在于,其对葡萄糖-6-磷酸和烟酰胺腺嘌呤二核苷酸磷酸(NADP)的米氏常数降低,对底物类似物——2-脱氧葡萄糖-6-磷酸尤其是脱氨基NADP的利用率增加,热稳定性低,pH依赖性特征不同,在聚丙烯酰胺凝胶电泳中出现单一的G6PD活性条带。