The imbalance of chain synthesis in hemoglobin F.
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-gamma and pre-alpha zones of the chain separation chromatogram are considered to be modified gamma and alpha chains, respectively, and are included in calculating the gamm/alpha ratios for cord blood and sickle-cell blood. The gamma chain is labeled about 52% as efficiently as the alpha chain in reticulocytes. It is possible that nu chain synthesis is balanced in the bone marrow, but subsequently becomes unbalanced in the reticulocyte.
D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics; D005312:Fetal Blood / Q000378:metabolism; D005319:Fetal Hemoglobin / Q000235:genetics*; D005914:Globins / Q000235:genetics; D006720:Homozygote; D006801:Humans; D007231:Infant, Newborn; D046911:Macromolecular Substances
D046911:Macromolecular Substances; D005914:Globins; D005319:Fetal Hemoglobin