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

doi:10.1182/blood.v94.5.1825

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

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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 language	English (US)
Pages (from-to)	1825-1826
Number of pages	2
Journal	Blood
Volume	94
Issue number	5
DOIs	https://doi.org/10.1182/blood.v94.5.1825
State	Published - Sep 1 1999
Externally published	Yes

ASJC Scopus subject areas

Biochemistry
Immunology
Hematology
Cell Biology

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Ameri, A., Fairbanks, V. F., Yanik, G. A., Mahdi, F., Thibodeau, S. N., McCormick, D. J., Boxer, L. A., & McDonagh, K. T. (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, 94(5), 1825-1826. https://doi.org/10.1182/blood.v94.5.1825

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. https://doi.org/10.1182/blood.v94.5.1825

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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.",

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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.

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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.

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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

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