Hemoglobin Istanbul: substitution of glutamine for histidine in a proximal histidine (F8(92) ).
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Aksoy M, Erdem S, Efremov GD, Wilson JB, Huisman TH, Schroeder WA, Shelton JR, Shelton JB, Ulitin ON, Muftuoglu A
Hemoglobin Istanbul: substitution of glutamine for histidine in a proximal histidine (F8(92) ).
J Clin Invest. 1972 Sep;51(9):2380-7.
- PubMed ID
- 4639022 [ View in PubMed]
- Abstract
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.