Oxidative phosphorylation differences between mitochondrial DNA haplogroups modify the risk of Leber's hereditary optic neuropathy

► MtDNA haplogroup J and H mutation carriers have a different risk of suffering LHON. ► Cybrids allow the analysis of the cellular and molecular bases for the risk of LHON. ► MtDNA haplogroup J and H cybrids show phenotypic differences in OXPHOS function. ► MtDNA levels determine OXPHOS capacity. ► MtDNA levels may be responsible for the association of LHON with mtDNA haplogroups.