The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated chloride channel that resides in the apical membrane of many epithelial cells. Channel opening requires phosophorylation of serine residues in an intracellular regulatory domain by protein kinase A and as the binding and hydrolysis of ATP by intracellular nucleotide binding domains. Besides conducting the chloride ion, CFTR also regulates the function of other membrane proteins, directly or indirectly, notably the outwardly rectifying chloride channel and the epithelial sodium channel. The disease cystic fibrosis is caused by mutations in CFTR, which can result in defective protein production, defective processing and degradation in the endoplasmic reticulum, or defective channel pore properties or gating properties.