Furthermore, as NF-kB and TLR4 are negatively regulated by the oxidation, specifically via S-glutathionylation, and given that their inhibition is overcome by the action of the de-glutathionylating enzyme GLRX1 (Reynaert et al., 2006; Chantzoura et al., 2010; Aesif et al., 2011), we also analyzed the cellular content of the redox enzymes GLRX1 and TRX1 in FRDA fibroblasts, to understand if a correlation between redox homeostasis and NF-kB signaling pathway might occur in this disease. This evidence concerns the gene NFKB1 and Friedreich ataxia.