Product Data SheetProduct Name:CAS No.: Cat. No.:MWt:Formula:Purity :Ivacaftor873054-44-5HY-13017392.49C24H28N2O3>98%Solubility:DMSO ≥75mg/mL Water<1.2mg/mL Ethanol ≥3.8mg/mLMechanisms: Pathways:Membrane Transporter/Ion Channel; Target:CFTR Biological Activity:Ivacaftor (VX770) is a potentiator of CFTR targeting G551DCFTR and F508delCFTR with EC50 ofIvacaftor(VX-770)isapotentiatorofCFTRtargetingG551D-CFTRandF508del-CFTRwithEC50of100 nM and 25 nM, respectively. IC50 Value: 25 nM (F508del-CFTR);100 nM (G551D-CFTR) [1] Target: F508del-CFTR/G551D-CFTR in vitro: In recombinant cells VX-770 increased CFTR channel open probability (P(o)) in both theF508del processing mutation and the G551D gating mutation. VX-770 also increased Cl(-) secretionin cultured human CF bronchial epithelia (HBE) carrying the G551D gating mutation on one alleleand the F508del processing mutation on the other allele by approximately 10-fold, to approximately50% of that observed in HBE isolated from individuals without CF [1]. in vivo: At day 28, in the group of subjects who received 150 mg of VX-770, the median change inthe nasal potential difference (in response to the administration of a chloride-free isoproterenolsolutio...References:[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 Nov 3;106(44):18825-30. [2].AccursoFJ,etal.EffectofVX-770[2]. Accurso FJ, et al. Effect of VX770 in persons with cystic fibrosis and the G551DinpersonswithcysticfibrosisandtheG551D-CFTRCFTRmutation. N Engl J Med. 2010 Nov 18;363(21):1991-2003. Caution: Not fully tested. For research purposes onlyMedchemexpress LLCwww.medchemexpress.com18Wilkinson Way, Princeton, NJ 08540,USAess.com Web: www.medchemexpress.comEmail: info@medchemexpr