Differences in relative mitochondria number and gene
expression were seen primarily in day 10 cells. Significant differences were seen in redox
status as evaluated by alamarBlue staining in newly isolated CD34+ cells. Mitochondria
mediate oxidative phosphorylation and apoptosis, both of which are known to be dysregulated
in differentiating erythrocytes from β°-thalassemia/Hb E patients. The evidence presented
here suggest that there are inherent differences in these cells as early as the
erythroid progenitor cell stage, and that maximum deficit is seen coincident with high levels
of globin gene expression.