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Research Article Free access | 10.1172/JCI107050

Hemoglobin istanbul: substitution of glutamine for histidine in a proximal histidine (F8(92)β)

M. Aksoy, S. Erdem, G. D. Efremov, J. B. Wilson, T. H. J. Huisman, W. A. Schroeder, J. R. Shelton, J. B. Shelton, O. N. Ulitin, and A. Müftüoğlu

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Aksoy, M. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Erdem, S. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Efremov, G. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Wilson, J. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Huisman, T. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Schroeder, W. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Shelton, J. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Shelton, J. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Ulitin, O. in: PubMed | Google Scholar

Section of Hematology, Second Internal Clinic of the Istanbul Medical School, Capa, Istanbul, Turkey

Laboratory of Protein Chemistry, Medical College of Georgia, Augusta, Georgia 30902

Division of Chemistry and Chemical Engineering, California Institute of Technology, Pasadena, California 91109

First Medical Clinic, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey

Find articles by Müftüoğlu, A. in: PubMed | Google Scholar

Published September 1, 1972 - More info

Published in Volume 51, Issue 9 on September 1, 1972
J Clin Invest. 1972;51(9):2380–2387. https://doi.org/10.1172/JCI107050.
© 1972 The American Society for Clinical Investigation
Published September 1, 1972 - Version history
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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 β-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 β-chain was used.

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