–46 The two most common mitochondrial optic neuropathies are autosomal dominant optic atrophy associated with pathogenic variants in OPA1 and LHON with three mtDNA variants (m.3460G>A, m.11778G>A and m.14484T>C) accounting for ∼90% of cases. Here, OPA1 is linked to Leber hereditary optic neuropathy.