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]. This evidence concerns the gene NFE2L2 and frontotemporal dementia.