Name: Ivacaftor (VX-770)
Price: 228.00 USD

- Overview
  - Please contact us at for specific academic pricing.
    
    Background
    
    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
- Properties
  - Categories
    
    Potent CFTR potentiator
    
    Alternative Name
    
    N-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide
    
    CAS Number
    
    873054-44-5
    
    Molecular Formula
    
    C24H28N2O3
    
    Molecular Weight
    
    392.49
    
    Appearance
    
    A solid
    
    Purity
    
    99.41%
    
    Solubility
    
    insoluble in H2O; ≥19.6 mg/mL in DMSO; ≥2.95 mg/mL in EtOH with ultrasonic
    
    Storage
    
    Store at -20°C
    
    SMILES
    
    CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C
    
    * For Research Use Only
- Reference
  - 1. Phua YL, Chen KH, et al. "Loss of miR-17~92 results in dysregulation of Cftr in nephron progenitors." Am J Physiol Renal Physiol. 2019 May 1;316(5):F993-F1005. PMID:30838872