In particular, the mitochondrial DNA (mtDNA) primary mutation, m.11778G>A/MT-ND4 (p.R340H), pathogenic for Leber’s hereditary optic neuropathy (LHON), induced a reduction in rotenone sensitivity to its inhibitory effect in vitro [17]. This evidence concerns the gene MT-ND4 and Leber hereditary optic neuropathy.