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Ivacaftor (VX-770)Potent CFTR potentiator

Ivacaftor (VX-770)

Catalog No. A5047
Size Price Stock Qty
10mM (in 1mL DMSO) $80.00 In stock
Evaluation Sample $28.00 In stock
5mg $70.00 In stock
25mg $250.00 In stock
100mg $650.00 In stock

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Sample solution is provided at 25 µL, 10mM.

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Related Biological Data

Ivacaftor inhibits mutant CFTR.

Biological Activity

Description Ivacaftor (VX-770) is a potentiator of CFTR for G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.
Targets G551D-CFTR F508del-CFTR        
IC50 100 nM 25 nM        

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Chemical Properties

Cas No. 873054-44-5 SDF Download SDF
Chemical Name N-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide
Canonical SMILES CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C
Formula C24H28N2O3 M.Wt 392.49
Solubility Soluble in DMSO > 10 mM Storage Store at -20°C
General tips For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.Stock solution can be stored below -20℃ for several months.
Shipping Condition Evaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request


Ivacaftor (VX-770) is a potent and orally bioavailable small molecule potentiator of cystic fibrosis transmembrane conductance regulator (CFTR). Studies in both G551D- and F508del-CFTR expressing cells have shown VX-700 combined with forskolin, but not VX-700 alone, has significantly increased CFTR-mediated Cl-secretion. The EC50 values of VX-700 in G551D- and F508del-CFTR are 100 nM and 25 nM, respectively [1].

VX-770 has been reported to reduce ENaC-mediated Na+ absorption and increase the amount of fluid on the apical surface in human CF bronchial epithelia (HBE) carrying G551D/F508del [1]. VX-770 combined with OAG has been shown to increase G551D-CFTR activity and OAG-dependent Ca2+ influx and in Chinese hamster ovary (CHO) cells [2].

1. Van Goor F1, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J,Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30.
2. Vachel L1, Norez C, Becq F, Vandebrouck C. Effect of VX-770 (ivacaftor) and OAG on Ca2+ influx and CFTR activity in G551D and F508del-CFTR expressing cells. J Cyst Fibros. 2013 Dec;12(6):584-91