Absence of beta-globin synthesis and excess of alpha-globin synthesis in homozygous beta-Thalassemia

Globin chain synthesis has been studied in β‐thalassemia by incubating red blood cells of 10 homozygous thalassemic patients with [3H] amino acids, and by measuring the incorporation of radioactivity into the globin chains, separated by column chromatography. Five of the ten patients were never transfused, five were polytransfused. The following results were obtained: (a) absence of β chain synthesis in both polytransfused and never trasfused patients; (b) excess of α chain synthesis in the ten cases examined (ratio α synthesis/(γ+δ) synthesis = 3.0). In three heterozygous subjects β globin synthesis and an excess of α chain synthesis was observed (ratio α/(β+γ+δ) = 1.6). The possible interpretations of these results are presented in the discussion. Copyright © 1967, Wiley Blackwell. All rights reserved