title: 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.
abstract: Several members of a Ugandan family were heterozygous for the gamma beta 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 alpha and Ggamma 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 Ggamma form of hereditary persistence of fetal haemoglobin rather than that of thalassaemia.
mesh_terms: D000595:Amino Acid Sequence; D000906:Antibodies; D002848:Chromatography, DEAE-Cellulose; D004590:Electrophoresis, Paper; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis* / Q000276:immunology; D006453:Hemoglobinopathies / Q000097:blood* / Q000235:genetics; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006579:Heterozygote; D006801:Humans; D007630:Kenya; D008297:Male; D010375:Pedigree; D014454:Uganda
chemical_list: D000906:Antibodies; D006455:Hemoglobins, Abnormal; D005319:Fetal Hemoglobin