Ivacaftor potentiates CFTR to restore chloride gating function in CFTR with class III gating defects, such as with the G551D variant. The star symbols on the CFTR-G551D protein icon and the CFTR-F508del protein icon indicate that they are active. The metabolism of ivacaftor and the elimination of its metabolites in the liver is shown. The figure also contains the mechanism of action of lumacaftor, which chaperones the protein folding of the CFTR-F508del and thereby restores correct localization of the protein, where ivacaftor can improve gating function. An interactive version is available at PharmGKB [https://www.pharmgkb.org/pathway/PA166153178].