Erythrocytosis Secondary to Increased Oxygen Affinity of a Mutant Hemoglobin, Hemoglobin Kempsey-Reference-Cited by-同舟云学术

Erythrocytosis Secondary to Increased Oxygen Affinity of a Mutant Hemoglobin, Hemoglobin Kempsey

Published:1968-05-01 Issue:5 Volume:31 Page:623-632
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

REED CON S.¹²,HAMPSON ROGER¹²,GORDON SUSAN¹²,JONES RICHARD T.¹³,NOVY MILES J.¹⁴,BRIMHALL BERNADINE¹⁵,EDWARDS MILES J.¹⁵,KOLER ROBERT D.¹⁵

Affiliation:

1. Department of Haematology, Sydney Hospital, Sydney, Australia and the Departments of Biochemistry and Medicine, University of Oregon Medical School, Portland, Oregon.

2. Sydney Hospital, Sydney, Australia.

3. University of Oregon Medical School, Portland, Oregon.

4. National Institute of Child Health aud Human Development; Present address, Department of Surgery, Boston University School of Medicine, Boston, Mass.

5. University of Oregon Medical School, Portland, Ore.

Abstract

Abstract An abnormal hemoglobin, termed hemoglobin Kempsey, was found in association with erythrocytosis in ten members of an Australian family. Genetic transmission fits an autosomal dominant pattern. Chemical characterization of hemoglobin Kempsey identified a single amino acid substitution, β99Asp→Asn. Properties of hemoglobin Kempsey include a marked increase in oxygen affinity, retention of the Bohr effect, and decreased heme-heme interaction. Possible structure-function relations of this and other abnormal hemoglobins associated with erythrocytosis are discussed.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/31/5/623/573806/623.pdf

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