Fig. S2.

UCM53 and UCM108 are more effective than the previously described compound.

FRDA patients-derived lymphoblasts cell lines FRDA 798 (A) and FRDA 214 (B) or lymphoblasts derived from the corresponding unaffected carrier siblings, FRDA 241 or FRDA 215 respectively, were cultured in the presence of 10 μM of the indicated UCM, or DMSO alone (contr) for 5 days. Total cell extracts were resolved on SDS–PAGE and analyzed with anti-frataxin antibody, or anti-tubulin, as a loading control. The graphs represent the relative frataxin abundance as quantified by densitometric analysis and normalized with tubulin levels. Data represent the mean ± SEM from three different independent experiments. Tub: tubulin; mat: mature frataxin.