Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), alpha 87 (F8) His -> Tyr: Evidence for an electrostatic model of alpha M hemoglobin assembly

Authors
Ameri, A Fairbanks, VF Yanik, GA Mahdi, F Thibodeau, SN McCormick, DJ Boxer, LA McDonagh, KT
Citation
A. Ameri et al., Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), alpha 87 (F8) His -> Tyr: Evidence for an electrostatic model of alpha M hemoglobin assembly, BLOOD, 94(5), 1999, pp. 1825-1826
Citations number
17
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
BLOOD
ISSN journal
00064971 → ACNP
Volume
94
Issue
5
Year of publication
1999
Pages
1825 - 1826
Database
ISI
SICI code
0006-4971(19990901)94:5<1825:IOTMGD>2.0.ZU;2-U
Abstract
We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, alpha 87 (F8) His --> Tyr] resides in the alpha 1-g lobin gene. The proportion of Hb M observed is higher than that predicted f or an alpha 1-globin variant. Our evidence suggests that the greater-than- expected proportion of Hb M-Kankakee results from preferential association of the electronegative beta-globin chains with the alpha(M)-globin chains t hat are more electropositive than normal alpha-globin chains. (C) 1999 by T he American Society of Hematology.