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已有文章阐明通过自由基形成、脂质过氧化和铁毒性的氧化损伤概念.氧化威胁在地中海贫血的溶血中起重要作用,本文旨在复习地中海贫血红细胞对氧化威胁敏感性增加的有关资料.珠蛋白链过量基因缺陷直接导致地中海贫血红细胞含过量血红蛋白亚单位,β-及α-地中海贫血分别积聚α及β珠蛋白链.已知在体外氧化作用下,这些不稳定血红蛋白产生氧自由基,如超氧化物及高效羟基.氧自由基起动氧化活动链,首先导致过量高铁血红蛋白形成,然后转变成可逆性和不可逆性的高铁血色原,高铁血色原易沉淀于细胞内,最后分解为血红素和珠蛋白,使变性珠蛋白链、血红素和铁充斥红细胞膜.过量的珠蛋白链在β-及α-地中海贫血中分别以单体α链及相对稳定的β四聚体存在,它们与不同膜蛋白结合,改变其正常结构和功能.
Articles have been published to elucidate the concept of oxidative damage through free radical formation, lipid peroxidation and iron toxicity.Oxidation threat plays an important role in the hemolysis of thalassemia and this article aims to review relevant data on the increased susceptibility of thalassemic erythrocytes to oxidative stress. Overexpression of globin chains leads directly to excess hemoglobin subunits in thalassemic erythrocytes and accumulation of alpha and beta globin chains, respectively, in beta- and alpha-thalassemias, It is known that these unstable hemoglobin produce oxygen free radicals under in vitro oxidation, Such as superoxide and highly effective hydroxyl.Oxygen radicals start oxidation chain, first lead to excessive formation of methemoglobin, and then converted into reversible and irreversible high-quality melasma, methemoglobin easily sedimentation in the cell, and finally broken down into heme And globin, erythrocyte membrane is filled with denatured globin chains, heme and iron.The excess of globin chain exists in the beta-and alpha-thalassemias as a single alpha chain and a relatively stable beta tetramer, respectively Different membrane proteins combine to change their normal structure and function.