Erythrocyte function and marrow regulation in hemoglobin Bethesda (β145 histidine)

John W. Adamson; Akira Hayashi; George Stamatoyannopoulos; Wilbur F. Burger

doi:10.1172/JCI107111

^{Hematology Service, Clinical Pathology Department, National Institutes of Health, Bethesda, Maryland 20014}

^{Malcolm Grow U. S. Air Force Medical Center, Andrews Air Force Base, Washington, D. C. 20331}

^{Division of Hematology, University of Washington School of Medicine, Seattle, Washington 98195}

^{Division of Medical Genetics, University of Washington School of Medicine, Seattle, Washington 98195}

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

^{Hematology Service, Clinical Pathology Department, National Institutes of Health, Bethesda, Maryland 20014}

^{Malcolm Grow U. S. Air Force Medical Center, Andrews Air Force Base, Washington, D. C. 20331}

^{Division of Hematology, University of Washington School of Medicine, Seattle, Washington 98195}

^{Division of Medical Genetics, University of Washington School of Medicine, Seattle, Washington 98195}

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

^{Hematology Service, Clinical Pathology Department, National Institutes of Health, Bethesda, Maryland 20014}

^{Malcolm Grow U. S. Air Force Medical Center, Andrews Air Force Base, Washington, D. C. 20331}

^{Division of Hematology, University of Washington School of Medicine, Seattle, Washington 98195}

^{Division of Medical Genetics, University of Washington School of Medicine, Seattle, Washington 98195}

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

^{Hematology Service, Clinical Pathology Department, National Institutes of Health, Bethesda, Maryland 20014}

^{Malcolm Grow U. S. Air Force Medical Center, Andrews Air Force Base, Washington, D. C. 20331}

^{Division of Hematology, University of Washington School of Medicine, Seattle, Washington 98195}

^{Division of Medical Genetics, University of Washington School of Medicine, Seattle, Washington 98195}

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

Published in Volume 51, Issue 11 on November 1, 1972
J Clin Invest. 1972;51(11):2883–2888. https://doi.org/10.1172/JCI107111.
© 1972 The American Society for Clinical Investigation

Published November 1, 1972 - Version history

Hemoglobin Bethesda (β145 histidine) is one of the two mutants known to affect the penultimate hemoglobin tyrosines. The result of this substitution is extreme disorganization of the oxygenation function of the molecule. Red cells containing 45% Hb Bethesda and 55% Hb A have increased oxygen affinity but, paradoxically, a normal Bohr effect. As is usually seen with other hemoglobins with increased oxygen affinity, Hb Bethesda clinically is manifest in heterozygotes by erythrocytosis. Red cell production in affected individuals is erythropoietin dependent. The reciprocal interdependence of oxygen delivery and effective erythropoiesis was documented by alterations in erythropoietin excretion, quantitative iron kinetics, and reticulocyte production in response to phlebotomy-induced reduction in the oxygen-carrying capacity.

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