Other iPSC lines were generated from fibroblasts carrying homoplasmic double-mtDNA mutations in the MT-ND1 gene (m.4160T>C; p.Leu285Pro) and in the MT-ND6 gene (m.14484T>C; p.Met464Val) (in this case, triggering the LHON “plus” phenotype), along with fibroblasts harboring the most common homoplasmic mtDNA mutation in the MT-ND4 gene (m.11778G>C; p.Arg340His) [101]. This evidence concerns the gene MT-ND4 and Leber hereditary optic neuropathy.