A consequence of the FXN depletion in FRDA is the increase of oxidative stress, and the most credited pathogenic hypothesis is that the FXN-mediated impairment of the mitochondrial iron–sulfur cluster (ISC)-containing enzymes (respiratory chain complexes I–III and aconitase) contributes to the Fenton-mediated overproduction of ROS (Armstrong et al., 2010; Gomes and Santos, 2013; Vaubel and Isaya, 2013; Abeti et al., 2016; Lupoli et al., 2018). Here, FXN is linked to Friedreich ataxia.