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一种独特的电泳迁移缓慢的葡萄糖6-磷酸脱氢酶变体(旭川葡萄糖6-磷酸脱氢酶),其最适pH值明显呈酸性。

A unique electrophoretic slow-moving glucose 6-phosphate dehydrogenase variant (G6PD Asahikawa) with a markedly acidic pH optimum.

作者信息

Takizawa T, Fujii H, Takegawa S, Takahashi K, Hirono A, Morisaki T, Kanno H, Oka R, Yoshioka H, Miwa S

出版信息

Hum Genet. 1984;68(1):70-2. doi: 10.1007/BF00293876.

Abstract

A new glucose 6-phosphate dehydrogenase (G6PD) variant associated with chronic nonspherocytic hemolytic anemia was discovered in Japan. The patient showed hemolytic crises after upper respiratory infections. The enzyme activity was about 3.8% of the normal. The partially purified enzyme revealed slow anodal electrophoretic mobility, high Km NADP, marked thermal-instability, and increased affinity for a substrate analogue (deamino-NADP). A particular characteristic of this enzyme was a biphasic pH curve with a greatly increased activity at low pH values. From these results, this variant was clearly different from hitherto observed G6PD variants, and was designated G6PD Asahikawa.

摘要

在日本发现了一种与慢性非球形细胞溶血性贫血相关的新型葡萄糖-6-磷酸脱氢酶(G6PD)变体。该患者在上呼吸道感染后出现溶血危象。酶活性约为正常水平的3.8%。部分纯化的酶显示出缓慢的阳极电泳迁移率、高Km NADP、明显的热不稳定性以及对底物类似物(脱氨基-NADP)的亲和力增加。这种酶的一个特殊特征是具有双相pH曲线,在低pH值时活性大大增加。根据这些结果,该变体与迄今观察到的G6PD变体明显不同,被命名为G6PD旭川。

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