Ivacaftor (VX-770)

Catalog No.S1144

Ivacaftor (VX-770) Chemical Structure

Molecular Weight(MW): 392.49

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

Size Price Stock Quantity  
In DMSO USD 112 In stock
USD 170 In stock
USD 320 In stock
USD 970 In stock
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Cited by 27 Publications

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Biological Activity

Description Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
Features The first potent and orally available CFTR potentiator to enter human clinical trials.
Targets
F508del-CFTR [1]
(Fisher rat thyroid cells)
G551D-CFTR [1]
(Fisher rat thyroid cells)
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 Lines Assay Type Concentration Incubation Time Formulation Activity Description PMID
HBE  NFLKdVNHfW6ldHnvckBCe3OjeR?= MUmxNEDPxE1? NHvxVpoyOCCvaX6= NVzhfo01[XWpbXXueJMhS0[WUj3k[ZBmdmSnboSgbY9vKHS{YX7zdI9zfMLi M3Xm[VI1OTB4OECx
CFBE41o- M3rCR2Z2dmO2aX;uJGF{e2G7 MVGxNEDDvU1? MUHpcoR2[2W|IILvZpV{fCCrbnPy[YF{\XNiaX6gZY5qd25idILhcpNxd3K2 NILt[YYzOjd4OEGzNC=>
HBE  MULGeY5kfGmxbjDBd5NigQ>? MkTzNVAhyrWP NFzlVXdifWevZX70d{BETlSULXTldIVv\GWwdDDhcolwdiC2cnHud5BwenRiYXP0bZZqfHl? NF;PbI8zOjd4OEGzNC=>
HBE  M1fBbWZ2dmO2aX;uJGF{e2G7 MV2xNEDDvU1? NF3lOGIzPCCq NGjkN2JqdmS3Y3XzJIEhdW:mZYP0JIJ2fCC|aXfubYZq[2GwdDDpcoNz\WG|ZTDpckBCW0xiZHXweIg> M2XLOVIzPzZ6MUOw
HBE  NXS0VYZtTnWwY4Tpc44hSXO|YYm= MVyxNEDDvU1? M3rXOZBwfGWwdHnheIV{KEOIVGKt[IVx\W6mZX70JGl{[yxicnXnZZJldGW|czDv[kBxemmxcjDh[I1qdmm|dILheIlwdiCxZjDDV2U> NHLESWYzOjd4OEGzNC=>
HBE  NHPTSG5HfW6ldHnvckBCe3OjeR?= NVS2N|duOTBiwsXN MWjwZZJ1cWGubImgdoV{fG:{ZYOg[IVxdGW2aX;uJI9nKEGVTDDk[ZB1cCCrbjDDV2UhfHKnYYTl[EBud26xbHH5[ZJ{ NF35N3ozOjd4OEGzNC=>

... Click to View More Cell Line Experimental Data

Assay
Methods Test Index PMID
Western blot
PPARγ / pERK; 

PubMed: 30498130     


Caco-2 cells incubated with P31–43 in the presence or absence of VX-770. Immunoblot of PPARc or phospho-ERK1/2 (phERK1/2) and densitometric analysis of protein levels relative to b-actin.

NLRP3; 

PubMed: 30498130     


Immunoblotting with specific antibodies (NLRP3) in Caco-2 cells challenged for 2 or 4 h in the presence or absence of VX-770. NLRP3 expression.

Rδf508; 

PubMed: 25101887     


Effect of VX-770 on the expression pattern of low temperature-rescued ΔF508-CFTR-3HA determined by immunoblot. Cells were treated with VX-770 alone for 24 hours at 26°C. CFTR was visualized with anti-HA antibody, and anti–Na+/K+-ATPase antibody served as loading control. 

30498130 25101887
Immunofluorescence
F-actin; 

PubMed: 30498130     


Confocal image staining with anti-F-actin. DAPI (blue), nuclear counterstaining. Scale bar, 50 μm.

30498130

Protocol

Solubility (25°C)

In vitro DMSO 78 mg/mL (198.73 mM)
Water Insoluble
Ethanol 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 Information

Molecular Weight 392.49
Formula

C24H28N2O3

CAS No. 873054-44-5
Storage powder
in solvent
Synonyms N/A

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Clinical Trial Information

NCT Number Recruitment Conditions Sponsor/Collaborators Start Date Phases
NCT03251911 Not yet recruiting Chronic Bronchitis University of Alabama at Birmingham January 1 2020 Phase 4
NCT03251911 Not yet recruiting Chronic Bronchitis University of Alabama at Birmingham January 1 2020 Phase 4
NCT03439865 Not yet recruiting Chronic Rhinosinusitis (Diagnosis) University of Alabama at Birmingham|National Heart Lung and Blood Institute (NHLBI) July 2019 Early Phase 1
NCT03439865 Not yet recruiting Chronic Rhinosinusitis (Diagnosis) University of Alabama at Birmingham|National Heart Lung and Blood Institute (NHLBI) July 2019 Early Phase 1
NCT03783286 Recruiting Cystic Fibrosis Children''s Hospital of Philadelphia|Vertex Pharmaceuticals Incorporated February 6 2019 --
NCT03783286 Recruiting Cystic Fibrosis Children''s Hospital of Philadelphia|Vertex Pharmaceuticals Incorporated February 6 2019 --

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CFTR Signaling Pathway Map

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Cell Lines Assay Type Concentration Incubation Time Formulation Activity Description PMID