Previous investigations have revealed that more than 90% of LHON cases are related to one of three primary point mutations in the mitochondrial DNA (mtDNA): m.11778G>A, m.3460G>A, and m.14484T>C, which encode the ND4, ND1, and ND6 subunits of Complex I in the mitochondrial respiratory chain, respectively (5–7). This evidence concerns the gene MT-ND6 and Leber hereditary optic neuropathy.