Ivacaftor-d19 (CAS 1413431-22-7)

Ivacaftor-d19 serves as an internal standard for the accurate quantification of ivacaftor in analytical chemistry, particularly during gas chromatography (GC) or liquid chromatography-mass spectrometry (LC-MS) procedures. Ivacaftor is recognized for its interaction with the cystic fibrosis transmembrane conductance regulator (CFTR), facilitating improved chloride transport. This compound may act to significantly enhance the CFTR-mediated epithelial current in cells bearing the G551D missense mutation, a genetic variation closely linked with cystic fibrosis, by approximately 4 times (EC50 = 100 nM) in the presence of forskolin. However, it does not influence the current in cells lacking forskolin stimulation. Additionally, ivacaftor has been observed to augment chloride secretion in human cystic fibrosis bronchial epithelial cells that have both the G551D mutation and the prevalent ΔF508 processing mutation. Its mechanism of action involves direct binding to the CFTR, leading to the opening of the CFTR channel through an ATP-independent process.