May A, Huehns E R
Br J Haematol. 1975 Jun;30(2):177-84. doi: 10.1111/j.1365-2141.1975.tb00532.x.
Oxygen dissociation studies were carried out on haemoglobin E (Hb E) at both high and low haemoglobin concentrations. Oxygen affinities of fresh red cells from three people homozygous for Hb E and from one with Hb E-beta thalassaemia (Hb-E trait/beta-thal trait) were low in three out of four patients studied, while the oxygen affinity of red cells from an individual with Hb-E was normal 2,3-DPG concentration in the fresh cells from the people with homozygous Hb E or Hb-E trait/beta-thal trait which showed low oxygen affinities were elevated sufficiently to account for the shifts observed. When the cells from two of these people with homozygous Hb E were depleted of 2,3-DPG. their oxygen affinities became the same as that of similarly treated normal cells. Pure 'stripped' Hb E in dilute solution behaved identically to Hb A in respect of P50, Bohr shift, haem-haem interaction, and interaction with inorganic phosphate or 2,3-DPG. Hb E, therefore, has the same oxypgen dissociation properties as Hb A both in dilute solution and in the red cell. The low oxygen affinities found in the fresh cells and in whole blood are caused by high 2,3-DPG concentrations within the cell.
在高血红蛋白浓度和低血红蛋白浓度条件下均对血红蛋白E(Hb E)进行了氧解离研究。在研究的4例患者中,3例纯合子Hb E患者以及1例Hb E-β地中海贫血患者(Hb-E性状/β-地中海贫血性状)新鲜红细胞的氧亲和力较低,而1例Hb-E患者红细胞的氧亲和力正常。纯合子Hb E或Hb-E性状/β-地中海贫血性状患者新鲜细胞中表现出低氧亲和力者,其2,3-DPG浓度升高幅度足以解释所观察到的偏移。当其中2例纯合子Hb E患者的细胞去除2,3-DPG后,其氧亲和力与同样处理的正常细胞相同。在P50、波尔效应、血红素-血红素相互作用以及与无机磷酸盐或2,3-DPG相互作用方面,稀溶液中的纯“脱辅基”Hb E与Hb A表现相同。因此,无论是在稀溶液还是在红细胞中,Hb E都具有与Hb A相同的氧解离特性。新鲜细胞和全血中发现的低氧亲和力是由细胞内高浓度的2,3-DPG所致。