A second case regards NC_012920.1:m.14538 A > G, YP_003024037.1:p.Phe46Leu in MT-ND6, reported in the literature20 as a novel pathogenic LHON variant but classified as likely-benign by APOGEE 2 (score 0.08, probability = 0.002). This evidence concerns the gene MCAT and Leber hereditary optic neuropathy.