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Biological Activity for Ivacaftor
Ivacaftor is a potent and selective potentiator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) that targets F508del-CFTR and G551D-CFTR (EC50 values are 25 nM and 100 nM, respectively); its potentiation activity depends on the level of CFTR phosphorylation. Ivacaftor reversibly binds to wild type (WT-CFTR) and G551D-CFTR mutants, increasing the open probability via an ATP-independent mechanism, and enhances cAMP/PKA-signaling mediated gating activity for both WT-CFTR and G551D-CFTR mutants. In cultured human CF bronchial epithelia with G551D/F508del mutations, Ivacaftor increases Forskolin (Cat. No. 1099)-induced transepithelial current (EC50 = 236 nM) and increases chloride secretion by 10-fold. Also rescues endothelial CFTR expression and function and prevents endothelial barrier failure and protein leak in human pulmonary microvascular endothelial cells. Ivacaftor is orally bioavailable.
Technical Data for Ivacaftor
|Storage||Store at -20°C|
The technical data provided above is for guidance only. For batch specific data refer to the Certificate of Analysis.
Tocris products are intended for laboratory research use only, unless stated otherwise.
Solubility Data for Ivacaftor
|Solvent||Max Conc. mg/mL||Max Conc. mM|
Preparing Stock Solutions for Ivacaftor
The following data is based on the product molecular weight 392.5. Batch specific molecular weights may vary from batch to batch due to the degree of hydration, which will affect the solvent volumes required to prepare stock solutions.
|Concentration / Solvent Volume / Mass||1 mg||5 mg||10 mg|
|1 mM||2.55 mL||12.74 mL||25.48 mL|
|5 mM||0.51 mL||2.55 mL||5.1 mL|
|10 mM||0.25 mL||1.27 mL||2.55 mL|
|50 mM||0.05 mL||0.25 mL||0.51 mL|
References for Ivacaftor
References are publications that support the biological activity of the product.
Jih et al (2013) Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle. Proc.Natl.Acad.Sci.U.S.A. 110 4404 PMID: 23440202
Cui (2019) VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level. Sci.Rep. 9 13460 PMID: 31530897
Erfinanda et al (2022) Loss of endothelial CFTR drives barrier failure and edema formation in lung infection and can be targeted by CFTR potentiation. Sci.Transl.Med. 14 eabg8577 PMID: 36475904
Levring et al (2023) CFTR function, pathology and pharmacology at single-molecule resolution. Nature 616 606 PMID: 36949202
Eckford et al (2012) Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator VX-770 (Ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation dependent but ATP-independent manner. J.Biol.Chem. 287 36639 PMID: 22942289
Nguyen et al (2021) Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells. Sci.Rep. 11 904 PMID: 33441643
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Keywords: Ivacaftor, Ivacaftor supplier, Ivacaftor, Cystic, Fibrosis, Transmembrane, Conductance, Regulator, CFTR, potentiator, G551D, F508, 7887, Tocris Bioscience
Citations for Ivacaftor
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