Of note, a few dominant inherited OPA3 variants, p.G93S, p.Q105E, and p.V3_G4insAP result in a rare dominant disorder (ADOAC; MIM 165300) involving optic atrophy, cataracts and extrapyramidal signs [16], [17], [18]. Here, OPA3 is linked to Leber hereditary optic neuropathy.