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The Kenya Form of Hereditary Persistence of Fetal Haemoglobin: Structural Studies and Evidence for Homogeneous Distribution of Haemoglobin F using Fluorescent Anti‐haemoglobin F Antibodies
Author(s) -
Nute P. E.,
Wood W. G.,
Stamatoyannopoulos G.,
Olweny C.,
Failkow P. J.
Publication year - 1976
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1976.tb01875.x
Subject(s) - fetus , antibody , persistence (discontinuity) , phenotype , homogeneous , fluorescein isothiocyanate , fetal hemoglobin , heterozygote advantage , biology , immunology , distribution (mathematics) , fluorescence , medicine , microbiology and biotechnology , gene , genetics , allele , pregnancy , mathematical analysis , physics , geotechnical engineering , mathematics , quantum mechanics , engineering , thermodynamics
S ummary . Several members of a Ugandan family were heterozygous for the γβ fusion gene of Haemoglobin Kenya. Levels of Hb Kenya were significantly higher than those in subjects of previous reports, ranging from 20.68 to 23.35% of the total haemoglobin. The individuals had also 5–8% Hb F, consisting solely of α and Gγ chains. Investigation of the distribution of Hb F among the red cells of Hb Kenya heterozygotes, using monospecific antibodies absorbed against pure Hb Kenya and rendered fluorescent by conjugation with fluorescein isothiocyanate, showed the presence of fetal haemoglobin in all red cells. The data suggest that the phenotype of the Hb Kenya trait resembles that of the Gγ form of hereditary persistence of fetal haemoglobin rather than that of thalassaemia.