In this study, markedly increased levels of LC3 but almost no changes in the levels of p62 in cybrids carrying the m.4401A>G mutation suggested a general increase in the capacity of the mutant cells to generate autophagosomes (83), in contrast with the reduced levels of LC3 in mutant cell lines carrying the LHON-associated ND5 12338T>C mutation (36). This evidence concerns the gene SQSTM1 and Leber hereditary optic neuropathy.