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伴有蚕豆病的葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症新变异型的生化特征:G-6-PD比勒费尔德(作者译)

[Biochemical characterization of a new variant of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency with favism: G-6-PD Bielefeld (author's transl)].

作者信息

Gahr M, Bornhalm D, Schröter W

出版信息

Klin Wochenschr. 1977 Apr 15;55(8):379-84. doi: 10.1007/BF01488623.

Abstract

A new variant of G-6-PD with favism has been detected in a German family. After partial purification the enzyme was biochemically characterized. It revealed normal electrophoretic mobility, normal thermostability and a flat pH-activity curve. The Km-value for G-6-P was reduced, whereas this value was normal for NADP. The substrate analogues 2-desoxy G-6-P, Gal-6-p and deamino-NADP were utilized to increased rates. The biochemical data were compatible with the benign clinical course.

摘要

在一个德国家庭中检测到一种伴有蚕豆病的新型葡萄糖-6-磷酸脱氢酶(G-6-PD)变体。经部分纯化后,对该酶进行了生化特性分析。结果显示其具有正常的电泳迁移率、正常的热稳定性以及平坦的pH-活性曲线。其对葡萄糖-6-磷酸(G-6-P)的米氏常数(Km值)降低,而对烟酰胺腺嘌呤二核苷酸磷酸(NADP)的Km值正常。底物类似物2-脱氧葡萄糖-6-磷酸、半乳糖-6-磷酸和脱氨基NADP的利用率有所提高。这些生化数据与良性临床病程相符。

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