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HbS显示一些与β~(b→Val)有关的异常分子行为,知道最清楚的是,在低氧应激情况下,HbS有明显的多聚化倾向;其次,蛋白表面的电荷改变,影响四聚体的装配;再者,HbS不稳定带来的后果.这说明了镰形红细胞(RBC)病是氧化现象起病理作用的疾病之一.本文将对支持这种看法的资料以及对特异性镰形RBC膜成分氧化损害的资料加以综述.HbS的自身氧化脱氧Hb的生理氧合作用,涉及到一个来自Hb亚铁电子向氧的转运.氧合Hb倾向于以过氧化铁Hb方式存在.正常情况下,这一过程可以逆转.有时Hb允许氧以过氧化物形式分离,然后留下高铁Hb(metHb).因此,生理水平的metHb生成和内源性氧窘迫(Oxidant Stress).可因氧合HbS而加速.氧合HbS在37℃稀释液中孵育期间,自身氧化比HbA快1.7倍,HbS自身氧化对镰形RBC施加两个不寻常
HbS shows some abnormal molecular behavior related to β ~ (b → Val). What is most clear is that under hypoxic stress, HbS has obvious tendency of multimerization; secondly, the change of charge on the surface of protein affects four And the consequences of instability in HbS.This demonstrates that sickle cell disease (RBC) is one of the pathological causes of oxidative phenomena.This article will review the data supporting this view and the implications for specificity The data on the oxidative damage of the sickle-shaped RBC membrane components are summarized.His physiological oxygenation of HbS, which involves the transport of ferrous ions from Hb to oxygen, tends to exist in the form of ferric oxide Hb. Under normal circumstances, this process can be reversed.Hb sometimes allows oxygen to form as peroxides, leaving the high-speed rail Hb (metHb). Therefore, the physiological level of metHb production and endogenous oxygen stress (Oxidant Stress) Oxygenated HbS and accelerated oxygenated HbS in 37 ℃ dilution during incubation, its own oxidation faster than HbA 1.7 times, HbS self-oxidation on sickle-shaped RBC exert two unusual