Erythropoiesis and Hemoglobin Regulation: A journey from laboratory to disorders
- Publisher: Erasmus University Rotterdam
textabstractThe hematopoietic system provides one of the most attractive systems for studies on development at both levels of molecular characterization and systems biology. Among the single-gene related disorders, hemoglobinopathies ranked on top with the prevalence of 4.83% in the world population (Urbinati et al., 2006). These reasons underlined the need of discovering the mechanisms underlying hemoglobin regulation and erythropoiesis. This thesis describes a journey beginning with an attempt to establish an in vitro hematopoietic progenitor cell system, passing through the transcriptional complexes regulating general aspects of erythropoiesis including the regulation of globin gene expression. It ends with a proposal to select patients with β-thalassemia for hydroxyurea treatment.