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正常和突变型葡萄糖6-磷酸脱氢酶(G6PD)在G6PD缺乏的红细胞内的截留。

Entrapment of normal and mutant glucose 6-phosphate dehydrogenase (G6PD) within G6PD deficient erythrocytes.

作者信息

De Flora A, Morelli A, Benatti U

出版信息

Bibl Haematol. 1985(51):50-6. doi: 10.1159/000410227.

Abstract

G6PD-deficient erythrocytes (Mediterranean type of this enzyme disorder) were loaded with a) normal G6PD purified to homogeneity from human erythrocytes, b) G6PD Mediterranean purified from deficient granulocytes. The first set of experiments led to complete normalization of biochemical properties of erythrocytes, as assessed by evaluating their metabolic competence under steady state conditions and under oxidative stress as well. The second type of experiment allowed us to conclude that mutant G6PD is not appreciably destroyed within the affected erythrocytes.

摘要

葡萄糖-6-磷酸脱氢酶(G6PD)缺乏的红细胞(该酶紊乱的地中海类型)用以下物质进行了加载:a)从人红细胞中纯化至同质的正常G6PD,b)从缺乏该酶的粒细胞中纯化的地中海型G6PD。第一组实验使红细胞的生化特性完全恢复正常,这是通过评估其在稳态条件下以及氧化应激下的代谢能力来确定的。第二类实验使我们得出结论,突变型G6PD在受影响的红细胞内不会被明显破坏。

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