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本文报道在安徽蚌埠发现的一个Hb Queens家系。先证者的溶血液在pH8.6电泳中出现泳速相当于Hb G组的慢速血红蛋白区带。肽链分析证实此异常血红蛋白为α链异常。将异常α链经柱层析分离纯化后作指纹图,把指纹图的异常肽斑洗脱,用微量双偶合法作氨基酸顺序测定,发现异常肽斑为α34亮氨酸被精氨酸取代,由此证实此异常血红蛋白为Hb Queens(α_2~(34Leu→Arg)β_2)。
This article reports a Hb Queens family discovered in Bengbu, Anhui Province. Hemolytic fluid of proband appeared in pH8.6 electrophoresis swim in the slow hemoglobin band equivalent to HbG group. Peptide chain analysis confirmed that this abnormal hemoglobin is an abnormal α chain. The abnormal α chain was separated and purified by column chromatography fingerprints, fingerprints abnormal peptide elution, with a trace of the double coupling method for the determination of amino acid sequence and found that abnormal peptide spots α34 leucine replaced by arginine, This confirmed that this abnormal hemoglobin Hb Queens (α 2 ~ (34Leu → Arg) β_2).