Nrf2 is a transcription factor that is activated by oxidative stress and binds to the antioxidant response elements of target genes, including those involved in glutathione synthesis, elimination of ROS, detoxification of xenobiotics and drug transport.32 We measured the level of AhR and Nrf2 in basal conditions and found that both factors were higher in unaffected mutation carriers than in LHON affected and controls (Figure 5a). This evidence concerns the gene NFE2L2 and Leber hereditary optic neuropathy.