Kondo T
1st Department of Medicine, School of Medicine, Hokkaido University, Sapporo.
Rinsho Byori. 1990 Apr;38(4):355-9.
During the delivery of oxygen by erythrocytes, highly reactive oxygen species such as superoxide anion arise. The presence of reactive species damages the cell constituents. Glutathione (GSH) functions to repair cells when they are attacked by oxidative stress. GSH is synthesized in erythrocytes and glutathione disulfide (GSSG) is transported outside the cells to maintain a high GSH/GSSG ratio. The redox cycle of GSH by glutathione reductase and glutathione peroxidase is closely related to G6PD. Hereditary enzyme deficiency related to GSH metabolism, with hemolytic anemia has been reported. G6PD deficiency causes hemolytic anemia due to insufficiency of the redox cycle of GSH. Deficiency of GSH synthesizing enzymes or glutathione reductase also causes hemolysis. Pyrimidine 5'-nucleotidase deficiency causes hemolytic anemia even when there is a high concentration of GSH. Accumulation of nucleotides in red cells causes inhibition of G6PD activity.
在红细胞输送氧气的过程中，会产生超氧阴离子等高活性氧物种。活性物种的存在会损害细胞成分。当细胞受到氧化应激攻击时，谷胱甘肽（GSH）发挥修复细胞的作用。谷胱甘肽在红细胞中合成，二硫化谷胱甘肽（GSSG）被转运到细胞外以维持高GSH/GSSG比值。谷胱甘肽还原酶和谷胱甘肽过氧化物酶对谷胱甘肽的氧化还原循环与葡萄糖-6-磷酸脱氢酶（G6PD）密切相关。据报道，与谷胱甘肽代谢相关的遗传性酶缺乏症会导致溶血性贫血。G6PD缺乏由于谷胱甘肽氧化还原循环不足而导致溶血性贫血。谷胱甘肽合成酶或谷胱甘肽还原酶的缺乏也会导致溶血。即使谷胱甘肽浓度很高，嘧啶5'-核苷酸酶缺乏也会导致溶血性贫血。红细胞中核苷酸的积累会抑制G6PD活性。
