Figure 1.

Deferiprone targets aconitase enzyme. Aconitase inhibition triggered by deferiprone in (A) cultured skin fibroblasts from control (open square) and two Friedreich's ataxia patients after 7 d of culture. Time-dependent aconitase inhibition by 150 μM deferiprone in (B) control (open square) and frataxin-depleted (shRNA treated) SK-N-AS cells. Error bars correspond to 1 SE (n = 3); *** denotes p < 0.001; n.s. non significant. Experimental conditions as described under Methods.