Citations to this article

Erythrocyte function and marrow regulation in hemoglobin Bethesda (β145 histidine)
John W. Adamson, … , George Stamatoyannopoulos, Wilbur F. Burger
John W. Adamson, … , George Stamatoyannopoulos, Wilbur F. Burger
Published November 1, 1972
Citation Information: J Clin Invest. 1972;51(11):2883-2888. https://doi.org/10.1172/JCI107111.
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Research Article

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

Abstract

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.

Authors

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

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Total citations by year

Year: 2023 2015 1984 1980 1978 1977 1975 1974 1973 Total
Citations: 1 1 1 2 1 1 2 1 1 11
Citation information

Citations to this article (11)

Title and authors Publication Year
Systematic Assessment of Protein C-Termini Mutated in Human Disorders.
FitzHugh ZT, Schiller MR 		Biomolecules 2023
A Japanese Family with Congenital Erythrocytosis Caused by Haemoglobin Bethesda
S Tamura, T Tamura, H Gima, A Nishikawa, Y Okamoto, N Kanazawa, L Relvas, E Cunha, MF McMullin, C Bento 		Internal Medicine 2015
High Altitude and Man
JB West, S Lahiri 		1984
Congenital Erythrocytosis: a New Form Associated with an Erythropoietin-dependent Mechanism
WH Whitcomb, C Peschle, M Moore, R Nitschke, JW Adamson 		British Journal of Haematology 1980
Automatic Oxygen Equilibrium Determination and its Clinical Application — Recent Topics
K Imai, A Hayashi, T Suzuki 		Hemoglobin 1980
Oxygen Transport to Tissue — III
IA Silver, M Erecińska, HI Bicher 		1978
POLYCYTHAEMIA AND HIGH AFFINITY HAEMOGLOBINS
AD Stephens 		British Journal of Haematology 1977
Hemoglobin fort gordon or α2β2145 Tyr → Asp, a new high-oxygen-affinity hemoglobin variant
HB Kleckner, JB Wilson, JG Lindeman, PD Stevens, G Niazi, E Hunter, CJ Chen, TH Huisman 		Biochimica et Biophysica Acta (BBA) - Protein Structure 1975
Structural and functional study of Hb Nancy β 145 (HC 2) Tyr → Asp a high oxygen affinity hemoglobin
G Gacon, H Wajcman, D Labie, C Vigneron 		FEBS Letters 1975
Interaction of Human Hemoglobin with Haptoglobin or Antihemoglobin Antibody
T Sasazuki, H Tsunoo, H Nakajima, K Imai 		The Journal of biological chemistry 1974
Hemoglobin Brigham (α2Aβ2100 Pro→Leu). HEMOGLOBIN VARIANT ASSOCIATED WITH FAMILIAL ERYTHROCYTOSIS
JJ Lokich, WC Moloney, HF Bunn, SM Bruckheimer, HM Ranney 		Journal of Clinical Investigation 1973

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