Cellular regulation of fetal hemoglobin production.
In this paper, we put together several observations from studies in erythroid cultures that suggest that the major regulatory events determining Hb F and Hb A formation in the red cells are taken at the level of erythroid stem cells. We outlined the evidence that suggests that the program of Hb F expression changes during the differentiation of the primitive progenitors, known as burst-forming units, and we raised several possibilities on how this differentiation-dependent change in developmental programs could be accomplished. Although many questions remain still unanswered, the work done so far suggests that the mechanisms of regulation of Hb F at the cellular level can be successfully probed with existing methodologies. Delineation of the cellular mechanism of Hb F regulation is required in order to find out whether the therapeutic manipulation of Hb F in the patient with Cooley's anemia is possible or not.
D000818:Animals; D002454:Cell Differentiation; D002478:Cells, Cultured; D004920:Erythropoiesis; D005319:Fetal Hemoglobin / Q000096:biosynthesis* / Q000235:genetics; D005333:Fetus; D006412:Hematopoietic Stem Cells / Q000166:cytology* / Q000378:metabolism; D006441:Hemoglobin A / Q000096:biosynthesis; D006801:Humans; D008954:Models, Biological; D013789:Thalassemia / Q000097:blood
D006441:Hemoglobin A; D005319:Fetal Hemoglobin