In the year 2000, the human OPA1 gene came to attention, as heterozygous mutations were associated with dominant optic atrophy (DOA) (1, 2), a blinding disorder originally described by the Danish ophthalmologist Paul Kjer in 1959, which usually leads to optic atrophy in the first decade of life (3). This evidence concerns the gene OPA1 and Leber hereditary optic neuropathy.