The Imbalance of Chain Synthesis in Hemoglobin F

Abstract

The synthesis of Hb F has been found to be unbalanced in a number of conditions, including homozygous HPFH, sickle-cell anemia, and normal term cord bloods. In this study, the counts from the pre-γ and pre-α zones of the chain separation chromatogram are considered to be modified γ and α chains, respectively, and are included in calculating the γ/α ratios for cord blood and sickle-cell blood. The γ chain is labeled about 52% as efficiently as the α chain in reticulocytes. It is possible that γ chain synthesis is balanced in the bone marrow, but subsequently becomes unbalanced in the reticulocyte.