VX-770 (Ivacaftor) is a CFTR potentiator and has been shown to potentiate normal CFTR, as well as CFTR with G551D and F508-del mutations. Ivacaftor directly binds to the ion channel, causes conformation change and open the ion channel to improve chloride transportation. In primary cultured human CF bronchial epithelia (HBE) carrying the G551D and F508del CFTR mutations, Ivacaftor (10 ?M) potently increases the forskolin-stimulated Cl- secretion with an EC50 of 236 nM. 
VX-770 (Ivacaftor) is approved for cystic fibrosis patients with G551D mutation, and has shown efficacy in a patient with S549N mutation. 
|Solubility:||Up to 100 mM in DMSO|
|Synonyms:||VX-770, VX770, Ivacaftor, Kalydeco|
Shipping Condition: The product is shipped in a glass vial at ambient temperature.
Storage condition: For longer shelf life, store solid powder or DMSO solution at -20oC desiccated.
|1.||Van Goor F et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. , Proc Natl Acad Sci U S A. 2009; 106(44):18825-30. Pubmed ID: 19846789|
|2.||McGarry ME and Nielson DW. Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N. Chest. 2013; 144(4):1376-8. Pubmed ID: 24081349|