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本文报告在甘肃省临夏市发现一例HbG_(Taipeio)先证者男性,11岁,汉族,世居临夏市。Hb醋酸纤维素薄膜电泳出现慢泳异常Hb区带,位于HbA与HbA_2之间,属G组异常Hb。按Clegg法,将珠蛋白在羧甲基纤维素CM—23上层析解链,分离出慢速β~x链。β~x链胰蛋白酶酶解物指纹分析显示β~ATp3肽斑消失,β~ATp5左下方出现变异新肽斑。经用Chang氏微量DABITC/PITC双偶合法测定氨基酸顺序相氨基酸分析,证实β链第22位谷氨酸被甘氨酸所取代。因此确定为HbG_(Taipeio)β22 Glu→Gly,并对其遗传、病理、地理分布和民族分布等进行了讨论。
This article reports that a case of HbG_ (Taipeio) proband was found in Linxia City, Gansu Province, aged 11, Han nationality, and lived in Linxia City. The Hb band of HbA and HbA_2 was found in Hb cellulose acetate film electrophoresis, belonging to abnormal Gb in G group. According to the Clegg method, globin was resolved on a carboxymethylcellulose CM-23 and a slow β-x chain was isolated. The fingerprinting analysis of β-x tryptase hydrolyzate showed that the disappearance of β-ATp3 peptide spots and the variability of new peptide spots in the lower left of β-ATp5. Amino acid sequence analysis by amino acid sequence analysis using Chang’s DABITC / PITC dual-coupling assay confirmed that glutamic acid at position 22 of the β-chain was replaced by glycine. So it was identified as HbG_ (Taipeio) β22 Glu → Gly, and its genetic, pathological, geographical distribution and ethnic distribution were discussed.