Hemoglobin Kenya, the product of a gamma-beta fusion gene: studies of the family.
Abstract/
Recently, we reported the discovery of a new hemoglobin variant, Hb Kenya, in a
26-year-old Kenyan of the Nilotic Luo tribe [1]. Chemical characterization of the
non-a chain of this hemoglobin showed that the amino terminal segment has the
sequence of the y chain of fetal hemoglobin (Hb F) and the carboxy terminal
segment that of the /8 chain of Hb A. It was concluded that the non-a chain of
Hb Kenya is the product of a hybrid gene that has been formed by nonhomologous
crossing over within gene loci.
On the basis primarily of electrophoretic and hematological observations (A. G.
Kendall, unpublished), the propositus was originally diagnosed as a heterozygote
for Hb S and the hereditary persistence of Hb F (HPFH). Because of the discovery
of Hb Kenya in the propositus, a reevaluation of his condition and that of members
of his immediate family was highly desirable. This report describes the results of
these studies as well as additional chemical evidence to support the y-)3 structure
of the non-a chain of Hb Kenya.