In light of our findings, the mitochondrial abnormalities recently observed in the optic nerve and retina of constitutive Nr2f1-knockout/Nr2fl-HET mice (Bertacchi et al., 2019a) might be a direct consequence of NR2F1 altered function that could contribute to the optic atrophy observed in BBSOAS mouse models. Here, NR2F1 is linked to hereditary optic atrophy.