2-4 LHON is associated with mutations in mitochondrial DNA encoding subunits of the respiratory chain complex I, mainly the m.3460G > A, m.11778G > A and m.14484T > C mutations in the MT-ND1, MT-ND4 and MT-Nd6 genes, respectively.2,5,6. Here, MT-ND6 is linked to Leber hereditary optic neuropathy.