Blood, Vol. 94 No. 5 (September 1), 1999:
pp. 1825-1826
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
A. Ameri,
V.F. Fairbanks,
G.A. Yanik,
F. Mahdi,
S.N. Thibodeau,
D.J. McCormick,
L.A. Boxer, and
K.T. McDonagh
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.
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 
-globin chains with the M-globin chains that are more
electropositive than normal -globin chains.
