PPARGC1A and inborn mitochondrial metabolism disorder: Bezafibrate, as a pan-PPAR activator, could up-regulate the expression of Peroxisome proliferator-activated receptor gamma coactivator 1-alpha, promote mitochondrial biogenesis and improve mitochondrial deficiency of mitochondrial diseases.[38]Therefore, the PPAR signaling pathway might be a potential disease-modifying therapeutic target.