Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly

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

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-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 α(M)-globin chains that are more electropositive than normal α-globin chains.

Original languageEnglish (US)
Pages (from-to)1825-1826
Number of pages2
JournalBlood
Volume94
Issue number5
StatePublished - Sep 1 1999

Fingerprint

Hemoglobin M
Methemoglobin
Globins
Static Electricity
Molecular Biology
Electrostatics
Defects
Genes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr : Evidence for an electrostatic model of αM hemoglobin assembly. / Ameri, A.; Fairbanks, V. F.; Yanik, G. A.; Mahdi, F.; Thibodeau, S. N.; McCormick, D. J.; Boxer, L. A.; McDonagh, K. T.

In: Blood, Vol. 94, No. 5, 01.09.1999, p. 1825-1826.

Research output: Contribution to journalArticle

Ameri, A, Fairbanks, VF, Yanik, GA, Mahdi, F, Thibodeau, SN, McCormick, DJ, Boxer, LA & McDonagh, KT 1999, 'Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly', Blood, vol. 94, no. 5, pp. 1825-1826.
Ameri, A. ; Fairbanks, V. F. ; Yanik, G. A. ; Mahdi, F. ; Thibodeau, S. N. ; McCormick, D. J. ; Boxer, L. A. ; McDonagh, K. T. / Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr : Evidence for an electrostatic model of αM hemoglobin assembly. In: Blood. 1999 ; Vol. 94, No. 5. pp. 1825-1826.
@article{448ddc1a49d9430496356d61312d7467,
title = "Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an electrostatic model of αM hemoglobin assembly",
abstract = "We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-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 α(M)-globin chains that are more electropositive than normal α-globin chains.",
author = "A. Ameri and Fairbanks, {V. F.} and Yanik, {G. A.} and F. Mahdi and Thibodeau, {S. N.} and McCormick, {D. J.} and Boxer, {L. A.} and McDonagh, {K. T.}",
year = "1999",
month = "9",
day = "1",
language = "English (US)",
volume = "94",
pages = "1825--1826",
journal = "Blood",
issn = "0006-4971",
publisher = "American Society of Hematology",
number = "5",

}

TY - JOUR

T1 - Identification of the molecular genetic defect of patients with methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr

T2 - Evidence for an electrostatic model of αM hemoglobin assembly

AU - Ameri, A.

AU - Fairbanks, V. F.

AU - Yanik, G. A.

AU - Mahdi, F.

AU - Thibodeau, S. N.

AU - McCormick, D. J.

AU - Boxer, L. A.

AU - McDonagh, K. T.

PY - 1999/9/1

Y1 - 1999/9/1

N2 - We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-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 α(M)-globin chains that are more electropositive than normal α-globin chains.

AB - We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-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 α(M)-globin chains that are more electropositive than normal α-globin chains.

UR - http://www.scopus.com/inward/record.url?scp=0033199944&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033199944&partnerID=8YFLogxK

M3 - Article

C2 - 10477710

AN - SCOPUS:0033199944

VL - 94

SP - 1825

EP - 1826

JO - Blood

JF - Blood

SN - 0006-4971

IS - 5

ER -