In the context of C9orf72-associated ALS/FTD, where expanded GGGGCC (G4C2) repeats form nuclear RNA foci and produce toxic DPRs via repeat-associated non-AUG (RAN) translation, Nrf2 activation has been reported to reduce both RNA foci and DPR accumulation [34,101]. This evidence concerns the gene NFE2L2 and amyotrophic lateral sclerosis.