Ivacaftor (VX-770)

Catalog No.S1144

Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.

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Ivacaftor (VX-770) Chemical Structure

Ivacaftor (VX-770) Chemical Structure
Molecular Weight: 392.49

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Product Description

Biological Activity

Description Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
Targets F508del-CFTR [1]
(Fisher rat thyroid cells)
G551D-CFTR [1]
(Fisher rat thyroid cells)
IC50 25 nM(EC50) 100 nM(EC50)
In vitro Ivacaftor (10 μM) significantly increases the forskolin-stimulated Cl- secretion (IT) by ~4-fold with an EC50 of 100 nM in the recombinant Fisher rat thyroid (FRT) cells expressing G551D gating mutation of CFTR, and by ~6-fold with an EC50 of 25 nM in the recombinant cells expressing temperature-corrected F508del processing mutation of CFTR. Consistent with the increases in the forskolin-stimulated IT, Ivacaftor (10 μM) increases the open probability (Po) of G551D-, F508del-, and wild-type CFTR by ~6-fold, ~5-fold and ~2-fold, respectively, indicating that Ivacaftor acts directly on CFTR to increase its gating activity. In primary cultured human CF bronchial epithelia (HBE) carrying the G551D and F508del CFTR mutations, Ivacaftor (10 μM) potently increases the forskolin-stimulated IT by ~10-fold from 5% to a maximum level of 48% of that measured in non-CF HBE, with an EC50 of 236 nM displaying ~70-fold more potency compared with the commonly used CFTR potentiator genistein, which has an EC50 of 16 μM. In HBE with F508del homozygous CFTR, Ivacaftor causes a significant increase in the forskolin-stimulated IT with an EC50 of 22 nM, to a less extent from 4% to 16% of non-CF HBE compared with the effect in G551D/F508del HBE. Due to CFTR potentiation, Ivacaftor inhibits excessive ENaC-mediated Na+ and fluid absorption with an IC50 of 43 nM, and decreases the amiloride response, resulting in an increase in the surface fluid and cilia beat frequency (CBF) in G551D/F508del HBE. [1]
Cell Data
Cell LinesAssay TypeConcentrationIncubation TimeFormulationActivity DescriptionPMID
HBE NEXTV2xHfW6ldHnvckBCe3OjeR?=M1jmNVExKM7:TR?=M1nVb|ExKG2rbh?=MUPheYdu\W62czDDSnRTNWSncHXu[IVvfCCrb36geJJidnOyb4L0xsA>Mnz4NlQyODZ6MEG=
HBE NVrDSGJNTnWwY4Tpc44hSXO|YYm=NGT0coYyOCEEtV2=NIO3eIFifWevZX70d{BETlSULXTldIVv\GWwdDDhcolwdiC2cnHud5BwenRiYXP0bZZqfHl?MoP3NlI4PjhzM{C=
HBE Mk\DSpVv[3Srb36gRZN{[Xl?NXizcoI5OTBiwsXNNUSzfG1oOjRiaB?=M2LES4lv\HWlZYOgZUBud2Snc4SgZpV1KHOrZ37p[olk[W62IHnuZ5Jm[XOnIHnuJGFUVCCmZYD0bC=>NWXnTm9QOjJ5NkixN|A>
HBE NXXVNpdRTnWwY4Tpc44hSXO|YYm=MlzKNVAhyrWPM3TSVZBwfGWwdHnheIV{KEOIVGKt[IVx\W6mZX70JGl{[yxicnXnZZJldGW|czDv[kBxemmxcjDh[I1qdmm|dILheIlwdiCxZjDDV2U>MnjKNlI4PjhzM{C=
HBE NILWWJFHfW6ldHnvckBCe3OjeR?=MkH5NVAhyrWPM4O5XJBienSrYXzsfUBz\XO2b4Lld{Bl\XCuZYTpc44hd2ZiQWPMJIRmeHSqIHnuJGNUTSC2cnXheIVlKG2xbn;sZZlmenN?MlzaNlI4PjhzM{C=

... Click to View More Cell Line Experimental Data

In vivo
Features The first potent and orally available CFTR potentiator to enter human clinical trials.

Protocol(Only for Reference)

Kinase Assay: [1]

Ussing Chamber Recordings The effect of Ivacaftor on CFTR-mediated Cl- secretion is characterized by measuring the CFTR-mediated IT in chambers using recombinant Fisher rat thyroid (FRT) cells expressing G551D, or F508del CFTR. Cells are grown on Costar Snapwell cell culture inserts maintained at 37 °C before recording. The cell culture inserts are mounted into an Ussing chamber to record IT in the voltage-clamp mode (Vhold = 0 mV). For FRT cells, the basolateral membrane is permeabilized with 360 μg/mL Nystatin and a basolateral to apical Cl- gradient is established. The basolateral bath solution contains 135 mM NaCl, 1.2 mM CaCl2, 1.2 mM MgCl2, 2.4 mM K2HPO4, 0.6 mM KHPO4, 10 mM N-2-hydroxyethylpiperazine-N'-2-ethanesulfonic acid (Hepes), and 10 mM dextrose (titrated to pH 7.4 with NaOH). The apical NaCl is replaced by equimolar Na+ gluconate (titrated to pH 7.4 with NaOH). The addition of a maximally effective concentration of forskolin (10 μM) is used to stimulate IT in the presence of various concentrations of Ivacaftor. All recordings are digitally acquired using Acquire and Analyze software. EC50 values are determined from the concentration-response curve of the increase in forskolin-stimulated IT after application of Ivacaftor.

Conversion of different model animals based on BSA (Value based on data from FDA Draft Guidelines)

SpeciesMouseRatRabbitGuinea pigHamsterDog
Weight (kg)
Body Surface Area (m2)0.0070.0250.
Km factor36128520
Animal A (mg/kg) = Animal B (mg/kg) multiplied by  Animal B Km
Animal A Km

For example, to modify the dose of resveratrol used for a mouse (22.4 mg/kg) to a dose based on the BSA for a rat, multiply 22.4 mg/kg by the Km factor for a mouse and then divide by the Km factor for a rat. This calculation results in a rat equivalent dose for resveratrol of 11.2 mg/kg.

Rat dose (mg/kg) = mouse dose (22.4 mg/kg) ×  mouse Km(3)  = 11.2 mg/kg
rat Km(6)


[1] Van Goor F, et al. Proc Natl Acad Sci U S A, 2009, 106(44), 18825-18830.

Clinical Trial Information( data from http://clinicaltrials.gov, updated on 2016-07-30)

NCT Number Recruitment Conditions Sponsor
Start Date Phases
NCT02730208 Not yet recruiting Cystic Fibrosis Vertex Pharmaceuticals Incorporated July 2016 Phase 2
NCT02807415 Recruiting Cystic Fibrosis Hannover Medical School|Heidelberg University|University  ...more Hannover Medical School|Heidelberg University|University of Giessen June 2016 --
NCT02797132 Recruiting Cystic Fibrosis Vertex Pharmaceuticals Incorporated May 2016 Phase 3
NCT02742519 Recruiting Cystic Fibrosis Vertex Pharmaceuticals Incorporated April 2016 Phase 3
NCT02725567 Recruiting Cystic Fibrosis Vertex Pharmaceuticals Incorporated March 2016 Phase 3

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Chemical Information

Download Ivacaftor (VX-770) SDF
Molecular Weight (MW) 392.49


CAS No. 873054-44-5
Storage 3 years -20℃powder
2 years -80℃in solvent
Synonyms N/A
Solubility (25°C) * In vitro DMSO 78 mg/mL (198.73 mM)
Water <1 mg/mL
Ethanol <1 mg/mL
* <1 mg/ml means slightly soluble or insoluble.
* Please note that Selleck tests the solubility of all compounds in-house, and the actual solubility may differ slightly from published values. This is normal and is due to slight batch-to-batch variations.
Chemical Name N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide

Tech Support

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Cell Lines Assay Type Concentration Incubation Time Formulation Activity Description PMID
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