Finally, peroxisome proliferator activated receptor gamma coactivator-1-alpha (PGC-1α) could be impaired in CF since it represents the key regulator of mitochondria biogenesis, a control protein for the major transcription factors involved in β-oxidation, Krebs cycle and oxidative phosphorylation [8], and a central modulator of OxS through the enhancement of reactive oxygen species (ROS) detoxification enzyme [9]. This evidence concerns the gene PPARGC1A and cystic fibrosis.