Fig. 8.

Supplementation with antioxidants. NRVM cultures were supplemented with the indicated concentrations of N-Acetylcysteine (NAC) and Tiron and, after 7 days of frataxin depletion, mitochondria were stained with TMRM (A and C). The amount of enlarged mitochondria was measured with ImageJ (B and D). Graphs represent the relative number of enlarged mitochondria relative to non-treated FXN deficient cells values. Only 50 μM Tiron treatment shows a significant decrease in the number of enlarged mitochondria in FXN interfered cells. Scale bar = 30 μm. # indicates statistic significance to non-treated shFxn (p<0,05).