Chemical Structure : Ivacaftor
CAS No.: 873054-44-5
Catalog No.: PC-43325Not For Human Use, Lab Use Only.
Ivacaftor (VX-770, VX770) is a potent, orally bioavailable CFTR potentiator, increases G551D- and F508del CFTR-mediated Cl- secretion with EC50 of 100 nM.
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Ivacaftor (VX-770, VX770) is a potent, orally bioavailable CFTR potentiator, increases G551D- and F508del CFTR-mediated Cl- secretion with EC50 of 100 nM.
Ivacaftor (VX-770) increases CFTR channel open probability (Po) in both the F508del processing mutation and the G551D gating mutation in recombinant cells, increases the Po of G551D CFTR by 6-fold, the Po of F508del- and wild-type CFTR by 5-fold and 2-fold, respectively.
Ivacaftor (VX-770) also potentiates CFTR-mediated Cl− secretion in primary cultures of G551D/F508del HBE and F508del HBE.
Ivacaftor (VX-770) demonstrates potential for treatment of cystic fibrosis with certain mutations in CFTR gene (primarily the G551D mutation).
M.Wt | 392.4907 | |
Formula | C24H28N2O3 | |
Appearance | Solid | |
Storage |
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Solubility |
10 mM in DMSO |
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Chemical Name/SMILES |
3-Quinolinecarboxamide, N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxo- |
1. Van Goor F, et al. Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30.
2. Accurso FJ, et al. N Engl J Med. 2010 Nov 18;363(21):1991-2003.
3. Yu W, et al. J Biol Chem. 2011 Jul 15;286(28):24714-25.
4. Hadida S, et al. J Med Chem. 2014 Dec 11;57(23):9776-95.
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