Effect of 2,3-diphosphoglycerate on oxygen affinity of blood in sickle cell anemia

Samuel Charache; Santiago Grisolia; Adam J. Fiedler; Andre E. Hellegers

doi:10.1172/JCI106294

^{Department of Medicine of The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205}

^{Department of Biochemistry of the University of Kansas School of Medicine, Kansas City, Kansas 66103}

^{Department of Obstetrics and Gynecology of Georgetown University School of Medicine, Washington, D. C. 20007}

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^{Department of Medicine of The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205}

^{Department of Biochemistry of the University of Kansas School of Medicine, Kansas City, Kansas 66103}

^{Department of Obstetrics and Gynecology of Georgetown University School of Medicine, Washington, D. C. 20007}

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

^{Department of Medicine of The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205}

^{Department of Biochemistry of the University of Kansas School of Medicine, Kansas City, Kansas 66103}

^{Department of Obstetrics and Gynecology of Georgetown University School of Medicine, Washington, D. C. 20007}

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

^{Department of Medicine of The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205}

^{Department of Biochemistry of the University of Kansas School of Medicine, Kansas City, Kansas 66103}

^{Department of Obstetrics and Gynecology of Georgetown University School of Medicine, Washington, D. C. 20007}

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Published in Volume 49, Issue 4 on April 1, 1970
J Clin Invest. 1970;49(4):806–812. https://doi.org/10.1172/JCI106294.
© 1970 The American Society for Clinical Investigation

Published April 1, 1970 - Version history

Blood of patients with sickle cell anemia (SS) exhibits decreased affinity for oxygen, although the oxygen affinity of hemoglobin S is the same as that of hemoglobin A. SS red cells contain more 2,3-diphosphoglycerate (DPG) than normal erythrocytes. The oxygen affinity of hemolyzed red cells is decreased by added DPG, and hemolysates prepared from SS red cells do not differ from normal hemolysates in this regard. Reduction of oxygen affinity to the levels found in intact SS red cells required DPG concentrations in excess of those found in most SS patients. The same was true of oxygen affinity of patients with pyruvate kinase deficiency. Other organic phosphates, as well as inorganic ions, are known to alter the oxygen affinity of dilute solutions of hemoglobin. These substances, the state of aggregation of hemoglobin molecules, and cytoarchitectural factors probably play roles in determining oxygen affinity of both normal and SS red cells.