Moving from our previous data [8,11] and from several lines of evidence overall showing decreased NRF2 activity either in FXN knockdown cells and in multiple FRDA models [7,9,12], in this study we investigate the effectiveness of several compounds, some of which are already adopted in clinical trials [18,19,20,21,22,23,24], to induce the NRF2 pathway and their impact on frataxin expression. Here, NFE2L2 is linked to Friedreich ataxia.