Identification of the Molecular Genetic Defect of Patients With Methemoglobin M Kankakee (M-Iwate), 87 (F8) His → Tyr: Evidence for an Electrostatic Model of M Hemoglobin Assembly-Reference-Cited by-同舟云学术

Identification of the Molecular Genetic Defect of Patients With Methemoglobin M Kankakee (M-Iwate), 87 (F8) His → Tyr: Evidence for an Electrostatic Model of M Hemoglobin Assembly

Published:1999-09-01 Issue:5 Volume:94 Page:1825-1826
ISSN:1528-0020
Container-title:Blood
language:en
Short-container-title:

Author:

Ameri A.¹,Fairbanks V.F.¹,Yanik G.A.¹,Mahdi F.¹,Thibodeau S.N.¹,McCormick D.J.¹,Boxer L.A.¹,McDonagh K.T.¹

Affiliation:

1. From the Division of Pediatric Hematology-Oncology and the Department of Internal Medicine, University of Michigan, Ann Arbor, MI; and the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Abstract

We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, 87 (F8) His → Tyr] resides in the 1-globin gene. The proportion of Hb M observed is higher than that predicted for an 1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the M-globin chains that are more electropositive than normal -globin chains.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference17 articles.

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2. Studies on relationships between structure and function of hemoglobin M-Iwate.;Hayashi;J Biol Chem,1966

3. Oxygen equilibrium of hemoglobin M-Iwate.;Kikuchi;Biochim Biophys Acta,1964

4. Molecular pathology of human hemoglobins.;Perutz;Nature,1968

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