In vitro synthesis of hemoglobin and hemoglobin chains in the BFUe-derived colonies from persons with α- or β-thalassemia

Publisher Website
Google Scholar
Abstract
The synthesis of α and non-α chains of human hemoglobin (Hb) was studied in reticulocytes and in BFUe-derived cell colonies from patients with α chain or β chain deficiencies. The subjects included normal adults (αα/αα) with or without a β chain variant (Hb S, Hb Leslie) or an α chain variant (Hb G-Georgia); α-thalassemia-2 heterozygotes (α0α/αα) with an α chain variant (G-Georgia or G-Philadelphia); an α-thalassemia-1 heterozygote (α0α0/αα); α-thalassemia-2 homozygotes (α0α/α0α) with a β chain variant (Hb S), an α chain variant (G-Philadelphia), a Hb S homozygosity with Hb G-Philadelphia, or a Hb G-Philadelphia homozygosity; and three black β+ -thalassemia homozygotes. Data from reticulocyte in vitro synthesis analyses showed the expected deficiencies. However, similar analyses of the Hb synthesized in cell colonies (even from the black β-thalassemia homozygotes) gave (nearly) balanced ∑α/∑non-α ratios. It is speculated that this balanced synthesis is due to a most effective proteolysis in the immature erythroblast which rapidly removes free α or β chains. The levels of Hb F and Hb A2 were considerably increased in these proerythroblasts; a two- to threefold increase in the synthesis of Hb A2 was observed over that seen in the reticulocytes.

This publication has 21 references indexed in Scilit: