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[与葡萄糖-6-磷酸脱氢酶活性缺乏相关的溶血性贫血患者红细胞葡萄糖-6-磷酸脱氢酶的电泳及动力学特性]

[Electrophoretic and kinetic properties of the erythrocyte glucose-6-phosphate dehydrogenase of patients with hemolytic anemia related to a deficit in the activity of that enzyme].

作者信息

Cherniak N B, Batishchev A I, Lamzina N V, Tokarev Iu N, Alekseev G A

出版信息

Vopr Med Khim. 1977 Mar-Apr;23(2):166-71.

PMID:18839
Abstract

Partially purified glucose-6-phosphate dehydrogenase (G6PD) was studied in erythrocytes of patients with hereditary hemolytic anemia. Kinetic and electrophoretic properties of G6PD distinctly varied both in patients--homozygotes and in maternal heterozygotes. All the enzymes studied together with the decreased activity possessed the reduced Km value for NADP and G6P. Anomalous enzymes were shown to differ from normal ones by several patterns (pH-dependency, thermostability, % of the substrate analogues utilization, electrophoretic mobility etc). The mutant variant of G6PD was not described earlier. The anomalies variant was called "Kremenchug" to indicate the place of proband origination.

摘要

对患有遗传性溶血性贫血患者的红细胞中的部分纯化葡萄糖-6-磷酸脱氢酶(G6PD)进行了研究。G6PD的动力学和电泳特性在患者(纯合子)和母亲杂合子中均有明显差异。所有研究的酶活性降低,同时对NADP和G6P的Km值降低。异常酶在几种模式(pH依赖性、热稳定性、底物类似物利用率、电泳迁移率等)上与正常酶不同。G6PD的突变变体此前未被描述。该异常变体被称为“克雷门丘格”以表明先证者的起源地。

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