Emerging evidence indicates that CFTR does not only act as a pure ion channel, but also as a hub protein that orchestrates the proteostasis network (PN) and influence multiple cellular functions.7, 8 The dysfunction of this hub in CF deranges major mechanisms of cell physiology, both in primary epithelial cells biopsied from F508del-CFTR homozygous patients and in mice bearing an equivalent mutation in the mouse CFTR gene (CF mice). This evidence concerns the gene CFTR and cystic fibrosis.