The higher PGC1-α expression that we found only in LHON cells carrying the carrier nuclear background promotes mitochondrial biogenesis, allowing for alternative routes bypassing complex I impairment, as well as increasing the antioxidant machinery, globally compensating for the impaired OXPHOS (Giordano et al., 2014). The gene discussed is PPARGC1A; the disease is Leber hereditary optic neuropathy.