Decrease of Alpha‐Hasharon Globin in Beta‐Thalassaemia

Abstract

In time course experiments performed in subjects carrying Hb Hasharon and .beta.-thalassemia the synthesis of .alpha.A-, .alpha.Hasharon-, .beta.- and .gamma.-globin was determined. The .alpha.-globin molecules synthesized in excess were removed from the red cell cytoplasm (phenomenon characteristic of .beta.-thalassemia). The .alpha.Hasharon-globin molecules were removed at a faster rate than normal .alpha.-globin. The preferential removal of the mutant .alpha.-globin explains the reduced level of Hb Hasharon found in subjects carrying the gene for .beta.-thalassemia. Reductions below normal levels of other Hb variants occurring in the presence of the counterpart-globin thalassemia are probably determined through a similar mechanism.