[CFTR and cystic fibrosis, a 50-year long history]

The medical and scientific history of cystic fibrosis will have a lasting impact on human medicine. It will take 50 years of scientific experiments and medical observations, favored by advances in genetics, molecular biology and physiology to go from the ionic theory showing that this disease is the consequence of a generalized defect of the transepithelial transport of NaCl until the cloning of the CFTR gene in 1989. The discovery of the gene and its mutations, the description of the CFTR protein and its role in the disease have revolutionized the physiology and the pathophysiology of ionic transports in epithelial cells of the respiratory and digestive systems.