Ivacaftor (VX-770)

Catalog No.S1144

For research use only.

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.

Ivacaftor (VX-770) Chemical Structure

CAS No. 873054-44-5

Selleck's Ivacaftor (VX-770) has been cited by 183 publications

Purity & Quality Control

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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  NUXpS2NuTnWwY4Tpc44hSXO|YYm= MorsNVAh|ryP MVmxNEBucW5? M2TBdIF2\22nboTzJGNHXFJvZHXw[Y5l\W62IHnvckB1emGwc4DvdpTDqA>? NF\BeJg9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{NEGwOlgxOSd-MkSxNFY5ODF:L3G+
CFBE41o- MkHQSpVv[3Srb36gRZN{[Xl? NGjzc5AyOCEEtV2= M4XrUIlv\HWlZYOgdo9jfXO2IHnuZ5Jm[XOnczDpckBidmmxbjD0doFve3CxcoS= NWfuWJBFRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkK3OlgyOzBpPkKyO|Y5OTNyPD;hQi=>
HBE  NH3O[5NHfW6ldHnvckBCe3OjeR?= NWLz[nIxOTBiwsXN NHjxb|hifWevZX70d{BETlSULXTldIVv\GWwdDDhcolwdiC2cnHud5BwenRiYXP0bZZqfHl? MUe8ZUB1[XKpZYS9K39jdGGwazegbJJm\j1paIT0dJM7Ny:ydXLt[YQvdmOkaT7ucI0vdmmqLnfvek8zOjd4OEGzNEc,OjJ5NkixN|A9N2F-
HBE  MWXGeY5kfGmxbjDBd5NigQ>? M325SlExKML3TR?= MlzzNlQhcA>? M3OwN4lv\HWlZYOgZUBud2Snc4SgZpV1KHOrZ37p[olk[W62IHnuZ5Jm[XOnIHnuJGFUVCCmZYD0bC=> NHiwRZg9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{Mke2PFE{OCd-MkK3OlgyOzB:L3G+
HBE  MnG1SpVv[3Srb36gRZN{[Xl? MlPiNVAhyrWP M1nVPZBwfGWwdHnheIV{KEOIVGKt[IVx\W6mZX70JGl{[yxicnXnZZJldGW|czDv[kBxemmxcjDh[I1qdmm|dILheIlwdiCxZjDDV2U> NY[2bmZ7RGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkK3OlgyOzBpPkKyO|Y5OTNyPD;hQi=>
HBE  MWnGeY5kfGmxbjDBd5NigQ>? NUXJTod[OTBiwsXN M4jNfpBienSrYXzsfUBz\XO2b4Lld{Bl\XCuZYTpc44hd2ZiQWPMJIRmeHSqIHnuJGNUTSC2cnXheIVlKG2xbn;sZZlmenN? M{DLdFxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ{N{[4NVMxLz5{Mke2PFE{ODxxYU6=
mouse NIH-3T3 cells MoPNSpVv[3Srb36gZZN{[Xl? MUGzNEBucW6| MXnQc5RmdnSrYYTpc44hd2ZiaIXtZY4hS0[WUjDGOVA5\GWuIH31eIFvfCCneIDy[ZN{\WRiaX6gcY92e2ViTlnIMVNVOyClZXzsd{Bi\nSncjCzNEBucW6|IHL5JIZtfW:{ZYPj[Y51KH[xbITh[4Uhe2Wwc3nu[{BweHSrY3HsJIF{e2G7LDDFR|UxKD1iMD6wNFMh|ryPLh?= M1nCPVxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ3NESxNFE{Lz5{NUS0NVAyOzxxYU6=
human bronchial epithelial cells NFi5ZWhHfW6ldHnvckBie3OjeR?= MlXRVI91\W62aXH0bY9vKG:oIHj1cYFvKEOIVGKgSlUxQGSnbD;HOVUyTCCvdYThcpQhcW5iaIXtZY4h[nKxbnPobYFtKGWyaYTo[Yxq[WxiY3XscJMh[nliVYPzbY5oKGOqYX3i[ZJ{KHKnY3;y[Ilv\yC2ZXPocolyfWVuIFXDOVAhRSByLkCyNkDPxE1w MX68ZUB1[XKpZYS9K39jdGGwazegbJJm\j1paIT0dJM7Ny:ydXLt[YQvdmOkaT7ucI0vdmmqLnfvek8zPTR2MUCxN{c,OjV2NEGwNVM9N2F-
human CFBE41o cells M17wcWZ2dmO2aX;uJIF{e2G7 MW[xNEBucW6| NEXD[HNRd3SnboTpZZRqd25ib3[gR2ZVWiCINUC4[IVtKG23dHHueEApfW6tbn;3ckBwemmpaX6pJIV5eHKnc4Pl[EBqdiCqdX3hckBETkKHNEHvJINmdGy|IHnuZ5Vj[XSnZDDmc5IhOTBibXnud{BqdiCycnXz[Y5k\SCxZjDmc5J{c2:uaX6gcYVie3W{ZXSg[o9zKDdic3Xjd{BjgSC\RmCgbIFtcWSnIHHzd4F6NCCHQ{WwJF0hOC5zMk[g{txONg>? Ml;DQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjlzNEi3OlMoRjJ7MUS4O|Y{RC:jPh?=
human bronchial epithelial cells Ml3mSpVv[3Srb36gZZN{[Xl? M2f6[XBwfGWwdHnheIlwdiCxZjDoeY1idiCFRmTSJGY2ODimZXygcZV1[W62IHnuJIh2dWGwIHLyc45kcGmjbDDldIl1cGWuaXHsJINmdGy|IHL5JHV{e2mwZzDjbIFu[mW{czDy[YNwemSrbnegeIVkcG6rcYXlMEBGSzVyIE2gNE4zOzZizszNMi=> M3e4NVxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ3NESxNFE{Lz5{NUS0NVAyOzxxYU6=
HEK293 cells NFjaT3VHfW6ldHnvckBie3OjeR?= MVqxNEBucW6| NVrEUpYzWG:2ZX70bYF1cW:wIH;mJGNHXFJiR{W1NWQhdXW2YX70JEh2dmuwb4fuJI9zcWerbjmg[ZhxemW|c3XkJIlvKEiHS{K5N{Bk\WyuczDpcoN2[mG2ZXSg[o9zKDFyIH3pcpMhcW5icILld4Vv[2Vib3[g[o9ze2uxbHnuJI1m[XO3cnXkJIZweiB{IH3pcpMh[nliWV\QJIhidGmmZTDhd5NigSxiRVO1NEA:KDFwMzFOwG0v NVvBfllkRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkmxOFg4PjNpPkK5NVQ5PzZ|PD;hQi=>
NRK-49F cells MYHGeY5kfGmxbjDhd5NigQ>? MX\Jcohq[mm2aX;uJI9nKFSJRj3i[ZRiOS2rbnT1Z4VlKHSxdHHsJINwdGyjZ3XuJIFk[3WvdXzheIlwdiCrbjDyZZQhVlKNLUS5SkBk\WyuczygTWM2OCB;IESg{txONg>? MlHNQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjV2NkexOVcoRjJ3NE[3NVU4RC:jPh?=
DAOY cells NEnuc5ZyUFSVIHHzd4F6 NVztcmV7eUiWUzDv[kBx\WSrYYTybYMh[2GwY3XyJINmdGxibHnu[ZMhfG9iaXTlcpRq\nlibYXseIlxdGVib4Dwc5J1fW6rdHnld{Bnd3JiZIL1[{Bz\XC3coDvd4lv\zpiQ3;u[olzdWG2b4L5JJNkemWnbjDmc5IhTEGRWTDj[Yxtew>? NUDxOIxlRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
NB-EBc1 cells NXrvdml1eUiWUzDhd5NigQ>? M1vaS5FJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KEOxbn\pdo1ifG:{eTDzZ5Jm\W5iZn;yJG5DNUWEY{GgZ4VtdHN? NIHjN409[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
MG 63 (6-TG R) cells M3HneJFJXFNiYYPzZZk> NWLKNXVWeUiWUzDv[kBx\WSrYYTybYMh[2GwY3XyJINmdGxibHnu[ZMhfG9iaXTlcpRq\nlibYXseIlxdGVib4Dwc5J1fW6rdHnld{Bnd3JiZIL1[{Bz\XC3coDvd4lv\zpiQ3;u[olzdWG2b4L5JJNkemWnbjDmc5IhVUdiNkOgLFYuXEdiUjmgZ4VtdHN? MlHmQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
RD cells M3;Ne5FJXFNiYYPzZZk> MWTxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBEd26oaYLtZZRwenlic3Py[YVvKG[xcjDSSEBk\Wyucx?= M4DZSFxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
SK-N-MC cells M{XLW5FJXFNiYYPzZZk> NUHC[5RGeUiWUzDv[kBx\WSrYYTybYMh[2GwY3XyJINmdGxibHnu[ZMhfG9iaXTlcpRq\nlibYXseIlxdGVib4Dwc5J1fW6rdHnld{Bnd3JiZIL1[{Bz\XC3coDvd4lv\zpiQ3;u[olzdWG2b4L5JJNkemWnbjDmc5IhW0tvTj3NR{Bk\Wyucx?= NV7icIRuRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
Saos-2 cells MWTxTHRUKGG|c3H5 NF60PFJyUFSVIH;mJJBm\GmjdILpZ{Bk[W6lZYKgZ4VtdCCuaX7ld{B1dyCrZHXueIlngSCvdXz0bZBt\SCxcIDvdpR2dmm2aXXzJIZweiCmcoXnJJJmeHW{cH;zbY5oQiCFb37mbZJu[XSxcomgd4Nz\WWwIH\vdkBU[W:|LUKgZ4VtdHN? M4WzZVxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
Assay
Methods Test Index PMID
Western blot Rδf508 ; NLRP3 ; PPARγ / pERK 25101887 30498130
Immunofluorescence F-actin 30498130

Protocol (from reference)

Solubility (25°C)

In vitro

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

Chemical Information

Molecular Weight 392.49
Formula

C24H28N2O3

CAS No. 873054-44-5
Storage 3 years -20°C powder
2 years -80°C in solvent
Smiles CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C

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Clinical Trial Information

NCT Number Recruitment Interventions Conditions Sponsor/Collaborators Start Date Phases
NCT04254705 Not yet recruiting Procedure: Rectal Biopsy Cystic Fibrosis Universitaire Ziekenhuizen Leuven|Vertex Pharmaceuticals Incorporated|KU Leuven|University of Lisbon March 1 2020 Not Applicable
NCT03085485 Recruiting Drug: Ivacaftor 150 MG|Drug: Placebo Chronic Obstructive Pulmonary Disease|Chronic Bronchitis University of Alabama at Birmingham|National Heart Lung and Blood Institute (NHLBI)|Vertex Pharmaceuticals Incorporated March 16 2017 Phase 2
NCT02724527 Unknown status Drug: Cavosonstat|Drug: Placebo Cystic Fibrosis Nivalis Therapeutics Inc. April 2016 Phase 2
NCT02725567 Recruiting Drug: ivacaftor Cystic Fibrosis Vertex Pharmaceuticals Incorporated March 2016 Phase 3

(data from https://clinicaltrials.gov, updated on 2022-01-17)

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