Aguilar i Bascompte J L, Wajcman H, Poyart C, Labie D
Nouv Rev Fr Hematol (1978). 1981;23(5):267-73.
A new variant, Hb Barcelona (beta 94 Asp leads to His) found in a Spanish family presenting with a mild polycythemia, is described. Its increase in oxygen affinity is explained by the rupture of the salt-bridge between beta 94 Asp and beta 146 His which normally stabilizes the deoxystructure and accounts for approximately half of the Bohr effect. A comparison between the estimated oxygen transport to the tissues by the blood from a patient with Hb Kempsey and that of the patients with Hb Barcelona showed, in the latter cases, that an almost normal oxygen delivery was obtained by the increase of the hemoglobin level.
本文描述了在一个患有轻度红细胞增多症的西班牙家庭中发现的一种新变体——血红蛋白巴塞罗那变体（β94位天冬氨酸突变为组氨酸）。其氧亲和力的增加是由于β94位天冬氨酸与β146位组氨酸之间盐桥的断裂，该盐桥通常稳定脱氧结构并构成约一半的波尔效应。对血红蛋白肯普西患者血液与血红蛋白巴塞罗那患者血液向组织输送氧气的估计进行比较后发现，在血红蛋白巴塞罗那患者中，通过提高血红蛋白水平可实现几乎正常的氧气输送。
