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[β-地中海贫血患者红细胞中某些酶活性降低的相关方面]

[Aspects of the decrease of some enzyme activities in erythrocytes of subjects with beta-thalassemia].

作者信息

Pescarmona G P, Sartori M L, Bosia A

出版信息

Boll Soc Ital Biol Sper. 1979 Apr 30;55(8):809-15.

PMID:554655
Abstract

G6PD, GSH-Px and 6PGD activity was found to be elevated in beta-thalassemia heterozygous erythrocytes. Estimates of the activities of the three enzymes in red cell fractions of differing mean age separated by centrifugation through a density gradient of Ficoll-Triosil layers, showed that the rate of in vivo decline was normal for G6PD and GSH-Px, and decreased for 6PGD. The increased enzyme activity results from increased rate of synthesis for G6PD and GSH-Px, and from higher in vivo stability for 6PGD.

摘要

研究发现,β地中海贫血杂合子红细胞中的葡萄糖-6-磷酸脱氢酶(G6PD)、谷胱甘肽过氧化物酶(GSH-Px)和6-磷酸葡萄糖酸脱氢酶(6PGD)活性升高。通过在Ficoll-三碘苯甲酸盐层密度梯度中离心分离不同平均年龄的红细胞组分,对这三种酶的活性进行评估,结果显示G6PD和GSH-Px的体内下降速率正常,而6PGD的下降速率降低。酶活性增加是由于G6PD和GSH-Px的合成速率增加,以及6PGD在体内具有更高的稳定性。

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