ROLE OF HYBRID TETRAMER FORMATION IN GELATION OF HEMOGLOBIN-S
ROLE OF HYBRID TETRAMER FORMATION IN GELATION OF HEMOGLOBIN-S
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DOI：
10.1038/256667a0
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发表时间：
1975-01-01
期刊：
影响因子：
64.8
通讯作者：
BALAZS, T
中科院分区：
文献类型：
--
作者：
BOOKCHIN, RM;NAGEL, RL;BALAZS, T
含有血红蛋白（Hb）S的红细胞从柔软的双凹面圆盘转变为坚硬的尖的“镰状”形式，这是镰状细胞病病理学的基础，这是由于这种突变血红蛋白在脱氧时聚合并产生细胞内凝胶的趋势。在镰状细胞病的遗传变异中，红细胞中存在的不同类型和比例的非S血红蛋白与Hb S一起在确定镰状化趋势的程度和疾病的严重程度方面很重要。含有Hb S沿着大量胎儿血红蛋白（Hb F，α2γ2）的红细胞，比含有类似比例的正常成人型Hb A（α2β2）的红细胞更不容易镰状化。这种差异可以用血红蛋白混合物溶液在体外通过测量最小胶凝浓度（MGC）来证明，所述最小胶凝浓度是在完全脱氧时混合物将形成凝胶的总血红蛋白的最小浓度。与S-A混合物相比，Hb S-F混合物的MGC更高，这与前一种组合在红细胞内的镰状化趋势较小相关。
THE transformation of erythrocytes containing haemoglobin (Hb) S from pliable biconcave disks into rigid pointed “sickled” forms, which underlies the pathology of sickle cell disease, results from the tendency of this mutant haemoglobin to polymerise and produce an intracellular gel on deoxygenation. In the genetic variants of sickle cell disease, the different types and proportions of non-S haemoglobins present in the red cells together with Hb S are important in determining the extent of the sickling tendency and the severity of the disease. Red cells containing Hb S along with a substantial proportion of foetal haemoglobin (Hb F, α2γ2), sickle less readily than cells having a similar proportion of the normal adult type, Hb A (α2β2). Such differences can be demonstrated with solutions of haemoglobin mixturesin vitroby measuring the minimum gelling concentrations (MGC), that is, the minimum concentration of total haemoglobin at which the mixture will form a gel on complete deoxygenation. The higher MGC of Hb S–F mixtures compared with S–A mixtures correlates with the lesser sickling tendency of the former combination within red cells.