Aksoy M, Erdem S, Efremov G D, Wilson J B, Huisman T H, Schroeder W A, Shelton J R, Shelton J B, Ulitin O N, Müftüoğlu A
J Clin Invest. 1972 Sep;51(9):2380-7. doi: 10.1172/JCI107050.
A presumably spontaneous mutation has resulted in the formation of Hemoglobin (Hb) Istanbul in which glutamine is substituted for histidine in the proximal position of the beta-chain (F8(92)). The anemia and other physiological effects that occur in the presence of Hb Istanbul were much ameliorated by splenectomy. Hb Istanbul is a relatively unstable molecule which produces a rather moderate case of "unstable hemoglobin hemolytic anemia."In the determination of structure, a method of preferential cleavage of an aspartyl-proline bond at residues 99-100 of the beta-chain was used.
一种可能的自发突变导致了血红蛋白（Hb）伊斯坦布尔的形成，其中β链近端位置（F8(92)）的组氨酸被谷氨酰胺取代。脾切除术后，Hb伊斯坦布尔存在时出现的贫血和其他生理效应得到了很大改善。Hb伊斯坦布尔是一种相对不稳定的分子，会引发较为中度的“不稳定血红蛋白溶血性贫血”。在结构测定中，使用了一种优先切割β链第99 - 100位天冬氨酰 - 脯氨酸键的方法。
