Combined these results suggest a complex association between mitochondria and the pathology
of β°-thalassemia/Hb E as mediated by erythroid differentiation. The results suggest that
there is a markedly different redox state in newly isolated CD34+ cells, and this may result
from the differing levels of EPO in these patients as compared to normal controls. Effects on
mitochondria as seen by mitotracker staining are seen by day 7 of differentiation, and significant
deficits in activity are seen on day 10, coincident with significant levels of globin chain
synthesis [7]. These results would suggest that the deposition of unpaired globin chains is
directly affecting the integrity of mitochondria. As there are more mitochondria present in
cells from thalassemia patients on day 10, it suggests that the effect is magnified, with the damage
to mitochondria at this point in time being co-incident with the onset of apoptosis in ineffective
erythropoiesis [