OPA1 and autosomal dominant optic atrophy: Although, the main pathological feature of ADOA is optic nerve degeneration and visual loss during early childhood, several patients develop multi-systemic deteriorations, including deafness, ataxia, myopathies and paraplegia among others, underlining the pivotal role of OPA1 activity in the maintenance of mitochondrial metabolism and tissue homeostasis (Yu-Wai-Man et al., 2010; Belenguer and Pellegrini, 2013).