Thus, in FRDA models, the activation of the Nrf2 pathway with omav is able to increase the availability of substrates for Complex I and protect the mitochondrial respiration, highlighting its beneficial role in mitochondrial bioenergetics (Dinkova-Kostova et al., 2015; Bartolome et al., 2017). This evidence concerns the gene NFE2L2 and Friedreich ataxia.