The balance between biogenesis and mitophagy is perturbed using in vitro models of DS, with hyperactivation of proteins from the rapamycin (mTOR) pathway responsible for mitophagy and impaired activity of PGC-1α pathway responsible for biogenesis (Valenti et al., 2016; Bordi et al., 2019; Mollo et al., 2019, 2020). This evidence concerns the gene PPARGC1A and Dravet syndrome.