A recent study highlighted the crucial role of mitochondrial defects in Drosophila models of C9orf72 ALS/FTD, suggesting that mitochondrial oxidative stress is a relevant mechanistic contributor to C9orf72 pathogenesis, leading to mitochondrial dysfunction and indicating the KEAP1/NRF2 signaling pathway as a key therapeutic target [130]. The gene discussed is KEAP1; the disease is amyotrophic lateral sclerosis.