Wajcman H, Promé D, Préhu C, Déon C, Riou J, Bouanga J C, Papassotiriou I, Lahary A, Galactéros F
Department of Biochemistry and INSERM U91, Hôpital Henri Mondor, Créteil, France.
Hemoglobin. 1998 Mar;22(2):129-40. doi: 10.3109/03630269809092137.
Hb Les Andelys [alpha83(F4)Leu-->Pro] is a mildly unstable variant that was found during glycated hemoglobin measurement in a French family. In this hemoglobin molecule the affected site, in the alpha chain, and the amino acid substitution are identical to those of Hb Santa Ana, an unstable beta chain variant. The structural abnormality was demonstrated by protein chemistry methods, involving, in addition to the classical techniques, a selective precipitation of the abnormal hemoglobin by isopropanol and a mass spectrometry analysis of the alphaT-9 peptide following carboxypeptidase digestion. DNA sequencing demonstrated that the mutation was CTG-->CCG at codon 83 of the alpha2 gene.
Hb莱斯昂代利斯[α83(F4)亮氨酸→脯氨酸]是一种轻度不稳定的变体，在一个法国家庭的糖化血红蛋白测量过程中被发现。在这种血红蛋白分子中，α链上的受影响位点和氨基酸替代与不稳定的β链变体Hb圣安娜相同。通过蛋白质化学方法证实了结构异常，除了经典技术外，还包括用异丙醇选择性沉淀异常血红蛋白以及对羧肽酶消化后的αT-9肽进行质谱分析。DNA测序表明，α2基因第83密码子处的突变是CTG→CCG。
