These results were consistent with those in cells carrying the deafness-associated 12S rRNA 1555A>G, tRNAPhe 593T>C or LHON-associated tRNAAla 5587T>C mutations (28, 29, 58) and but contrast with the upregulation of ubiquitination-dependent mitophagy but downregulation of ubiquitination-independent mitophagy in the cells carrying the LHON-associated ND1 3460G>A or ND6 14484T>C mutation (59, 60). Here, MT-ND6 is linked to Leber hereditary optic neuropathy.