Globin-chain synthesis in Hb H disease: the activity of red cell precursors and their mRNA

Globin-chain synthesis was determined in bone marrow and blood of four patients with Hb H disease and in a cell-free system supplemented with poly(A)-rich RNA from the patients. The relative synthesis of alpha-globin ranged between 0.36 and 0.49 in erythroid precursors and between 0.09 and 0.28 in reticulocytes. The relative quantity of biologically active alpha-globin mRNA in poly(A)-rich RNA from bone marrow and blood of these patients ranged between 0.09 and 0.21. The findings suggest the operation, within alpha-thalassemic bone marrow cells, of a translational control mechanism and/or the instability of alpha-globin mRNA.