In contrast, overexpression of PGC-1α, which facilitates mitochondrial biogenesis, has been found to prevent cell apoptosis.248 Increased mitochondrial biogenesis is thought to contribute to the phenomenon of incomplete penetrance in LHON and serves as a compensatory mechanism to restore mitochondrial turnover in LHON carriers.249 Thus, balancing mitochondrial biogenesis and mitophagy could represent a potential therapeutic target. Here, PPARGC1A is linked to Leber hereditary optic neuropathy.