Decrease of alpha-Hasharon globin in beta-thalassaemia.
In time course experiments performed in subjects carrying haemoglobin Hasharon and beta-thalassaemia the synthesis of alphaA-, alphaHasharon-, beta- and gamma-globin has been determined. The alpha-globin molecules synthesized in excess were found to be removed from the red cell cytoplasm (phenomenon characteristic of beta-thalassaemia, described by Bargellesi et al, 1968a); in addition the alphaHasharon-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 haemoglobin Hasharon found in subjects carrying the gene for beta-thalassaemia (Alberti et al, 1975). Reductions below normal levels of other haemoglobin variants occurring in the presence of the counterpart-globin thalassaemia are probably determined through a similar mechanism.
D005796:Genes; D006441:Hemoglobin A / Q000096:biosynthesis; D006455:Hemoglobins, Abnormal / Q000096:biosynthesis*; D006801:Humans; D066298:In Vitro Techniques; D009154:Mutation; D013789:Thalassemia / Q000097:blood* / Q000235:genetics; D013997:Time Factors
D006455:Hemoglobins, Abnormal; D006441:Hemoglobin A