The increase of basal autophagy and mitophagy is a common theme in mitochondrial diseases, in particular in those with optic atrophy, as documented by others and our own studies of different mtDNA mutations affecting ND subunits of complex I (Dombi et al., 2016; Granatiero et al., 2016), and OPA1-related syndromes (Carelli et al., 2015b; Liao et al., 2017). This evidence concerns the gene OPA1 and inborn mitochondrial metabolism disorder.