Even though over 50 variations in mtDNA are associated with LHON, 95% of cases are caused by one of three mtDNA missense changes: m.11778 G > A (60%) [4,8], m.14484 T > C (14%) [9,10,11], and m.3460 G > A (13%) [12,13] encoding for nicotinamide adenine dinucleotide ND4, ND1, and ND6 subunits of the respiration complex I, respectively [9,14]. This evidence concerns the gene MT-ND6 and Leber hereditary optic neuropathy.