Erythrocyte function and marrow regulation in hemoglobin Bethesda (beta-145 histidine)

The Journal of Clinical Investigation
J W AdamsonW F Burger


Hemoglobin Bethesda (beta145 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.


Jan 19, 1972·Nature: New Biology·A Hayashi, G Stamatoyannopoulos
Apr 28, 1971·Nature: New Biology·J Greer, M F Perutz
Jul 23, 1970·The New England Journal of Medicine·J TorranceC A Finch
Aug 31, 1968·Nature·M F Perutz, H Lehmann
Aug 1, 1969·The Journal of Clinical Investigation·J W AdamsonG Stamatoyannopoulos
Dec 1, 1956·A.M.A. Journal of Diseases of Children·J H JONXIS, H K VISSER
Dec 12, 1959·Nature·K BETKEI SCHLICHT

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Jun 1, 1977·British Journal of Haematology·A D Stephens
Aug 1, 1973·The Journal of Clinical Investigation·J J LokichH M Ranney
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