Oxidative stress, associated with a deficiency of the antioxidant factor NRF2, underlies the pathogenic mechanism in FRDA [16], and NRF2, besides its essential role as main regulator of redox homeostasis, is also a fine modulator of the inflammatory response, by interacting with the TLR4/NF-kB pathway. This evidence concerns the gene TLR4 and Friedreich ataxia.