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Research Article Free access | 10.1172/JCI107390

Hemoglobin Brigham (α2Aβ2100 Pro→Leu). HEMOGLOBIN VARIANT ASSOCIATED WITH FAMILIAL ERYTHROCYTOSIS

Jacob J. Lokich, William C. Moloney, H. Franklin Bunn, Sally M. Bruckheimer, and Helen M. Ranney

Division of Hematology of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Medicine, Boston, Massachusetts 02115

Department of Pediatrics, Boston, Massachusetts 02115

State University of New York at Buffalo, Buffalo, New York 14214

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

Division of Hematology of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Medicine, Boston, Massachusetts 02115

Department of Pediatrics, Boston, Massachusetts 02115

State University of New York at Buffalo, Buffalo, New York 14214

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

Division of Hematology of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Medicine, Boston, Massachusetts 02115

Department of Pediatrics, Boston, Massachusetts 02115

State University of New York at Buffalo, Buffalo, New York 14214

Find articles by Bunn, H. in: PubMed | Google Scholar

Division of Hematology of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Medicine, Boston, Massachusetts 02115

Department of Pediatrics, Boston, Massachusetts 02115

State University of New York at Buffalo, Buffalo, New York 14214

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

Division of Hematology of the Department of Medicine, Peter Bent Brigham Hospital, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Medicine, Boston, Massachusetts 02115

Department of Pediatrics, Boston, Massachusetts 02115

State University of New York at Buffalo, Buffalo, New York 14214

Find articles by Ranney, H. in: PubMed | Google Scholar

Published August 1, 1973 - More info

Published in Volume 52, Issue 8 on August 1, 1973
J Clin Invest. 1973;52(8):2060–2067. https://doi.org/10.1172/JCI107390.
© 1973 The American Society for Clinical Investigation
Published August 1, 1973 - Version history
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Abstract

Erythrocytosis associated with the presence of a hemoglobin with increased oxygen affinity has been reported for 10 hemoglobin variants, most of which demonstrate altered electrophoretic mobility. Several members of a family were found to have erythrocytosis, and both the whole blood and the hemoglobin exhibited increased oxygen affinity. Phosphate-free hemoglobin solutions had a normal Bohr effect and reactivity to 2,3-diphosphoglycerate. The electrophoretic properties of the hemoglobin were normal, but on peptide mapping of a tryptic digest of the isolated β-chains, a normal βT11 peptide and an abnormal βT11 with greater Rf were seen. Analysis of the abnormal peptide showed the substitution of leucine for the normal proline at β100 (helical residue G2).

The hemoglobin variant, designated Hb Brigham, serves to emphasize the necessity for detailed evaluation of the structure and function of hemoglobin in familial erythrocytosis even with electrophoretically “normal” hemoglobin.

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