Hemoglobin Rush [β101 (G3) Glutamine]: A New Unstable Hemoglobin Causing Mild Hemolytic Anemia-Reference-Cited by-同舟云学术

Hemoglobin Rush [β101 (G3) Glutamine]: A New Unstable Hemoglobin Causing Mild Hemolytic Anemia

Published:1974-02-01 Issue:2 Volume:43 Page:261-269
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Adams J. G.¹²,Winter W. P.¹³,Tausk K.¹⁴,Heller P.¹⁵

Affiliation:

1. Veterans Administration West Side Hospital, Chicago, Ill. 60680, and the Rush-Presbyterian-St. Luke’s Medical Center, Chicago, Ill. 60680.

2. Hemoglobin Research Laboratory Veterans Administration West Side Hospital, Chicago, Ill. 60680, and Assistant Professor of Genetics, Center for Genetics, University of Illinois College of Medicine, Chicago, Ill. 60612.

3. Department of Human Genetics, University of Michigan, Ann Arbor, Mich. 48104.

4. Rush Medical College, Chicago, Ill.

5. Veterans Administration West Side Hospital, Chicago, Ill. 60680 Professor of Medicine and Chief, Hematology Section, University of Illinois College of Medicine, Chicago, Ill. 60612.

Abstract

Abstract A mild hemolytic anemia in a 43-yr-old black woman was attributed to the presence of an abnormal hemoglobin (Hb Rush) which migrated cathodically to Hb A at pH 8.0. Its structural abnormality was found to be in the β-chain, β101 (G3) glu → gln. Another electrophoretic band at pH 8.0 proved to be a hybrid tetramer (αA2βAβRush). Hb Rush is heat unstable. A likely explanation of the instability is the presence of an uncovered positive charge in the central cavity where normally glutamic acid in position 101 neutralizes arginine in position 104 contributing to the net neutrality in this region. This neutrality is disturbed by the substitution of glutamic acid by glutamine in Hb Rush.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/43/2/261/578013/261.pdf

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