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在湖南地区发现两种类似的血红蛋白变异物——Hb 双峰(Hb Shuangfeng,简写 HbSF)〔α27(B8)Glu→Lys〕和 HbE〔β26(B8)GlU→Lys〕。前者不稳定,先证者表现感冒服药后溶血发作,后者却尚属稳定,杂合子无可见的临床表现。无疑这是研究结构与功能关系难得的两种天然变异物。本文报道此二种血红蛋白的结构、功能、合成及反应的动力学,从而探讨其不稳定和药物所致溶血的机理。采取正常人、HbSF 及 HbE 杂合子静脉血(草酸盐或肝素抗凝),用微晶纤维素薄层指纹技术证明其结构;用 DEAE-Sephadex A50 柱层分离纯化正常和异常血红蛋白;用自制压力计,按 Benesch 等描述不连续分光光度法作氧平衡曲线、P50、Hill 系数、Bohr 效应和变构
Two similar hemoglobin variants were found in Hunan Province - Hb Shuangfeng (HbSF) [α27 (B8) Glu → Lys] and HbE [β26 (B8) GlU → Lys]. The former is not stable, probands show hemorrhagic seizures after taking cold medicine, the latter is still stable, heterozygous no visible clinical manifestations. This is undoubtedly the study of the structure and function of two rare natural variants. This article reports the structure, function, synthesis and reaction kinetics of these two kinds of hemoglobin, so as to explore the mechanism of its instability and drug-induced hemolysis. Normal human HbSF and HbE heterozygous venous blood (oxalate or heparin anticoagulant) were taken and their structures were confirmed by microcrystalline cellulose thin-layer fingerprinting. Normal and abnormal hemoglobin were separated and purified by DEAE-Sephadex A50 column. Pressure gauges, as described by Benesch et al. Discontinuous spectrophotometry for oxygen balance curves, P50, Hill coefficient, Bohr effect and allosteric