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[葡萄糖-6-磷酸脱氢酶缺乏症患者血小板和红细胞中的蛋白质及磷脂]

[Proteins and phospholipids of thrombocytes and erythrocytes in glucose-6-phosphate dehydrogenase deficient patients].

作者信息

Spangenberg P, Heller R, Schwarzer E, Jacobasch G, Thielmann K, Till U

出版信息

Folia Haematol Int Mag Klin Morphol Blutforsch. 1984;111(6):774-81.

PMID:6083953
Abstract

Proteins and phospholipids of platelets and red cell membranes of glucose-6-phosphate-dehydrogenase-deficient patients with chronic hemolytic disease were investigated. The enzyme deficiency is not connected with alteration in the protein and phospholipid pattern of platelets. However, marked differences in the diamide-induced protein polymerization between normal and deficient platelets were found. Whereas normal platelets show only a weak polymerization which is reversible during diamide incubation of the cells, the deficient platelets demonstrate a considerable and long lasting protein polymerization. The results point to a direct connection between the thiol-disulfide status of platelets and intracellular level of glutathione, which is able to repair oxidative damages. Two of the six patients showed already protein polymers in their erythrocyte membrane without addition of any SH-oxidizing agent. The phospholipid pattern of the deficient red-cell membranes were comparable to the controls.

摘要

对患有慢性溶血性疾病的葡萄糖-6-磷酸脱氢酶缺乏症患者的血小板以及红细胞膜中的蛋白质和磷脂进行了研究。酶缺乏与血小板蛋白质和磷脂模式的改变无关。然而,发现正常血小板和缺乏症血小板在二酰胺诱导的蛋白质聚合方面存在显著差异。正常血小板仅表现出微弱的聚合,在细胞二酰胺孵育期间是可逆的,而缺乏症血小板则表现出相当程度且持久的蛋白质聚合。结果表明血小板的硫醇-二硫键状态与能够修复氧化损伤的谷胱甘肽细胞内水平之间存在直接联系。六名患者中有两名在未添加任何SH-氧化剂的情况下,其红细胞膜中就已经出现了蛋白质聚合物。缺乏症红细胞膜的磷脂模式与对照组相当。

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