Moving from those previous evidences showing a functional cross talk between Nrf2 and NF-kB pathways, in this study we analyzed the TLR4/NF-kB/IL-1β axis in fibroblasts of patients with FRDA, in order to understand if it could be modulated as a consequence of Nrf2 deficiency. Here, TLR4 is linked to Friedreich ataxia.