Indeed, since (i) the mitochondrial dysfunction and ROS generation are intricately related to changes in the glutathione redox system [147]; (ii) a drop of GSH levels is observed in CF cells [81,150], we studied more precisely GSH and GSH-dependent enzymes in order to trace back the link between mitochondrial dysfunction, low GSH levels and defective F508del-CFTR. The gene discussed is CFTR; the disease is cystic fibrosis.