Ivacaftor
Based on 29 publication(s) in Google Scholar
Ivacaftor (VX-770) is a potent and orally bioavailable CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively.
Nos produits utilisent uniquement pour la recherche. Nous ne vendons pas aux patients.
- Pureté: 99.97%
- CAS No.: 873054-44-5
- Formule: C24H28N2O3
- Masse moléculaire:392.49
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Stockage:Powder -20°C, 3 years , 4°C, 2 years ; In solvent -80°C, 1 year , -20°C, 6 months
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Activité biologique
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Solvant et solubilité
- Pureté et documentation
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Publications Citing Use of MedChemExpress (MCE) Ivacaftor
More- Acta Pharmacol Sin. 2025 Apr;46(4):1045-1057. [Abstract]
- Biomed Pharmacother. 2023 Oct:166:115399. [Abstract]
- JCI Insight. 2026 Mar 23;11(6):e198846. [Abstract]
- J Cyst Fibros. 2026 Jun 16:S1569-1993(26)01643-7. [Abstract]
- Am J Respir Cell Mol Biol. 2025 Mar;72(3):308-319. [Abstract]
- Cells. 2025 Oct 15;14(20):1601. [Abstract]
- Int J Mol Sci. 2026 Feb 23;27(4):2063. [Abstract]
- Int J Mol Sci. 2025 Aug 6;26(15):7618. [Abstract]
- J Mol Struct. 2025 Nov 10;1353:144673.
- Am J Physiol Cell Physiol. 2025 Mar 1;328(3):C856-C871. [Abstract]
- Front Cell Dev Biol. 2021 May 11:9:678209. [Abstract]
- Ther Adv Med Oncol. 2026 Jan 2:18:17588359251409010. [Abstract]
- Hum Gene Ther. 2024 Sep;35(17-18):695-709. [Abstract]
- Sci Rep. 2025 Apr 21;15(1):13767. [Abstract]
- Biomedicines. 2025 Jan 1;13(1):82. [Abstract]
- J Biol Chem. 2024 Nov;300(11):107873. [Abstract]
- Microbiol Spectr. 2025 Sep 2;13(9):e0227524. [Abstract]
- PNAS Nexus. 2025 Jul 4;4(7):pgaf211. [Abstract]
- J Cell Sci. 2022 Mar 1;135(5):jcs259002. [Abstract]
- Am J Physiol Lung Cell Mol Physiol. 2025 Aug 13. [Abstract]
- Org Process Res Dev. 2019, 23, 11, 2302-2322.
- Glycoconj J. 2025 Aug;42(3-4):173-186. [Abstract]
- Glycoconj J. 2025 Feb;42(1):1-14. [Abstract]
- bioRxiv. 2026 Jun 8:2026.06.01.729306. [Abstract]
- University of Lisbon. 2026.
- bioRxiv. 2025 Sep 11:2025.04.03.647093. [Abstract]
- bioRxiv. 2025 Nov 14:2024.10.30.620903. [Abstract]
- bioRxiv. 2024 August 16.
- University of Kentucky. 2022 Aug.
Customer Validation & Images
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Cell Imaging/Staining
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WB
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Flow Cytometry
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Cell Proliferation/Viability Assay
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Apoptosis Analysis
Activité biologique
EC50: 100 nM (G551D-CFTR), 25 nM (F508del-CFTR)[1]
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Cell Line
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Type | Value | Description | References |
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| Epithelial cell | EC50 |
0.236 μM
Compound: 48
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Potentiation of human CFTR F508del mutant in human bronchial epithelial cells by Ussing chambers recording technique
Potentiation of human CFTR F508del mutant in human bronchial epithelial cells by Ussing chambers recording technique
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[PMID: 25441013] |
| Epithelial cell | EC50 |
22 nM
Compound: 48
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Potentiation of human CFTR F508del/G551D mutant in human bronchial epithelial cells by Ussing chambers recording technique
Potentiation of human CFTR F508del/G551D mutant in human bronchial epithelial cells by Ussing chambers recording technique
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[PMID: 25441013] |
| NIH3T3 | EC50 |
3 nM
Compound: 48
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Potentiation of human CFTR F508del mutant expressed in mouse NIH-3T3 cells after 30 mins by fluorescent voltage sensing optical assay
Potentiation of human CFTR F508del mutant expressed in mouse NIH-3T3 cells after 30 mins by fluorescent voltage sensing optical assay
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[PMID: 25441013] |
| NRK-49F | IC50 |
4 μM
Compound: Ivacaftor
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Inhibition of TGF-beta1-induced total collagen accumulation in rat NRK-49F cells
Inhibition of TGF-beta1-induced total collagen accumulation in rat NRK-49F cells
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[PMID: 25467157] |
| Vero | CC50 |
27.34 μM
Compound: Ivacaftor
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Cell viability measured by CellTiter-Glo assay in Vero cells at MOI 0.05 after 72hr
Cell viability measured by CellTiter-Glo assay in Vero cells at MOI 0.05 after 72hr
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10.1101/2020.03.20.999730 |
| Vero | IC50 |
6.57 μM
Compound: Ivacaftor
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Antiviral activity against SARS-CoV-2 (viral titer) measured by plaque assay in Vero cells at MOI 0.0125 after 24 hr
Antiviral activity against SARS-CoV-2 (viral titer) measured by plaque assay in Vero cells at MOI 0.0125 after 24 hr
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10.1101/2020.03.20.999730 |
Ivacaftor (10?μM) increases the PC secretion activity by 3-fold for ABCB4-G535D, 13.7-fold for ABCB4-G536R, 6.7-fold for ABCB4-S1076C, 9.4-fold for ABCB4-S1176L, and 5.7-fold for ABCB4-G1178S. Ivacaftor corrects the functional defect of ABCB4 mutants[1]. Ivacaftor (10 μM) significantly increases CFTR activity in W1282X-expressing cells compared to R1162X CFTR cells[2]. Ivacaftor shows no significant activity against 160 targets tested including the GABAA benzodiazepine receptor. Ivacaftor increases the chloride secretion with an EC50 of 0.236 ± 0.200 μM, a 10-fold shift in potency compared to the F508del HBEs[3]. In recombinant cells, VX-770 increases CFTR channel open probability (Po) in both the F508del processing mutation and the G551D gating mutation. VX-770 increases forskolin-stimulated IT in temperature-corrected F508del-FRT cells by appr 6-fold with an EC50 of 25 nM[4].
MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.
MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.
Chemical Information
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CAS No. 873054-44-5
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Appearance Solid
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Masse moléculaire 392.49
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Formule C24H28N2O3
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Color White to off-white
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SMILES
CC(C)(C1=C(C=C(C(C(C)(C)C)=C1)O)NC(C2=CNC3=C(C2=O)C=CC=C3)=O)C
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Synonyms
VX-770
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Livraison
Room temperature in continental US; may vary elsewhere.
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Stockage
Powder -20°C 3 years 4°C 2 years In solvent -80°C 1 year -20°C 6 months
Publications (29)
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Journal Impact Factor
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Most Recent
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Acta Pharmacol Sin
Ivacaftor, a CFTR potentiator, synergizes with osimertinib against acquired resistance to osimertinib in NSCLC by regulating CFTR-PTEN-AKT axis. [Abstract]2025 Apr;46(4):1045-1057. PMID: 39627385 -
Biomed Pharmacother
CFTR potentiator ivacaftor protects against noise-induced hair cell loss by increasing Nrf2 and reducing oxidative stress. [Abstract]2023 Oct:166:115399. PMID: 37657258
Ivacaftor purchased from MedChemExpress. Usage Cited in: Biomed Pharmacother. 2023 Oct:166:115399. [Abstract]
Treatment with Ivacaftor (5 μM) prevents the decrease of CFTR and the increase of p-CFTR (S737) after noise exposure in OHCs.
Ivacaftor purchased from MedChemExpress. Usage Cited in: Biomed Pharmacother. 2023 Oct:166:115399. [Abstract]
Representative immunoblots show CFTR and p-CFTR (S737) with or without hydrogen peroxide or ivacaftor treatment versus DMSO alone (control). HEI-OC1 cells were pretreated with 5 μM Ivacaftor or equal concentration of DMSO for 12 h then treated with or without exposure to 10 mM hydrogen peroxide for 15 min.
Ivacaftor purchased from MedChemExpress. Usage Cited in: Biomed Pharmacother. 2023 Oct:166:115399. [Abstract]
ROS detection by DCFH-DA flow cytometry shows an increase in ROS accumulation after hydrogen peroxide stimulation of HEI-OC1 cells, while pretreatment with Ivacaftor (5 μM) blocks this effect.
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JCI Insight
Targeting PI3Kγ anchoring enhances CFTR membrane localization and modulator efficacy via PKD1. [Abstract]2026 Mar 23;11(6):e198846. PMID: 41869727 -
J Cyst Fibros
A functional comparison of vanzacaftor/tezacaftor/deutivacaftor and elexacaftor/tezacaftor/ivacaftor in patient-derived intestinal organoids with rare CFTR variants. [Abstract]2026 Jun 16:S1569-1993(26)01643-7. PMID: 42303492 -
Am J Respir Cell Mol Biol
Distinct Responses of Cystic Fibrosis Epithelial Cells to SARS-CoV-2 and Influenza A Virus. [Abstract]2025 Mar;72(3):308-319. PMID: 39311876 -
Cells
Mitochondrial Fragmentation Induced by the CFTR Modulators Lumacaftor and Ivacaftor in Immortalized Cystic Fibrosis Cell Lines. [Abstract]2025 Oct 15;14(20):1601. PMID: 41148816 -
Int J Mol Sci
Restoration of Defective CFTR in Human Nasal Respiratory Epithelial Cells by CFTR Modulators and mRNA Transfection. [Abstract]2026 Feb 23;27(4):2063. PMID: 41752197 -
Int J Mol Sci
Assessing CFTR Function and Epithelial Morphology in Human Nasal Respiratory Cell Cultures: A Combined Immunofluorescence and Electrophysiological Study. [Abstract]2025 Aug 6;26(15):7618. PMID: 40806745 -
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Am J Physiol Cell Physiol
Modeling ocular surface ion and water transport by generation of lipid- and mucin-producing human meibomian gland and conjunctival epithelial cells. [Abstract]2025 Mar 1;328(3):C856-C871. PMID: 39870373 -
Front Cell Dev Biol
2021 May 11:9:678209. PMID: 34046412
Ivacaftor purchased from MedChemExpress. Usage Cited in: Front Cell Dev Biol. 2021 May 11:9:678209. [Abstract]
The cells proliferation of GSC11 (left panel) and GBM1 (right panel) treated by 5 μM Ivacaftor.
Ivacaftor purchased from MedChemExpress. Usage Cited in: Front Cell Dev Biol. 2021 May 11:9:678209. [Abstract]
Ivacaftor (5 μM) treatment significantly increases cellular apoptosis in GSC11 and GBM1 cells determined by flow cytometry assay.
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Ther Adv Med Oncol
Repurposing of ivacaftor shows potential to treat ROR1 expressing high-grade serous ovarian cancer. [Abstract]2026 Jan 2:18:17588359251409010. PMID: 41487693 -
Hum Gene Ther
SP-101, A Novel Adeno-Associated Virus Gene Therapy for the Treatment of Cystic Fibrosis, Mediates Functional Correction of Primary Human Airway Epithelia From Donors with Cystic Fibrosis. [Abstract]2024 Sep;35(17-18):695-709. PMID: 39155805 -
Sci Rep
CFTR acts as a potential therapeutic target for attention deficit-hyperactivity disorder. [Abstract]2025 Apr 21;15(1):13767. PMID: 40258939 -
Biomedicines
2025 Jan 1;13(1):82. PMID: 39857666 -
J Biol Chem
A nonnatural peptide targeting the A-kinase anchoring function of PI3Kγ for therapeutic cAMP modulation in pulmonary cells. [Abstract]2024 Nov;300(11):107873. PMID: 39393573 -
Microbiol Spectr
Condition-dependent effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) on Aspergillus fumigatus growth. [Abstract]2025 Sep 2;13(9):e0227524. PMID: 40736245 -
PNAS Nexus
VX-445 (elexacaftor) inhibits chloride secretion across human bronchial epithelial cells by directly blocking KCa3.1 channels. [Abstract]2025 Jul 4;4(7):pgaf211. PMID: 40688096 -
J Cell Sci
Lipid-driven CFTR clustering is impaired in cystic fibrosis and restored by corrector drugs. [Abstract]2022 Mar 1;135(5):jcs259002. PMID: 35060604 -
Am J Physiol Lung Cell Mol Physiol
Effect of elexacaftor and bamocaftor on the metabolic and thermal stability of the F508del-CFTR protein in human airway epithelial cells. [Abstract]2025 Aug 13. PMID: 40803756 -
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Glycoconj J
Effects of GM1 ganglioside and its derivatives on ETI-rescued F508del-CFTR maturation and host-pathogen interactions in cystic fibrosis bronchial cells. [Abstract]2025 Aug;42(3-4):173-186. PMID: 40663265 -
Glycoconj J
Effect of CFTR modulators Elexacaftor/Tezacaftor/Ivacaftor on lipid metabolism in human bronchial epithelial cells. [Abstract]2025 Feb;42(1):1-14. PMID: 39797966 -
bioRxiv
2026 Jun 8:2026.06.01.729306. PMID: 42282787 -
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bioRxiv
General Trends in the Calnexin-Dependent Expression and Pharmacological Rescue of Clinical CFTR Variants. [Abstract]2025 Sep 11:2025.04.03.647093. PMID: 40964320 -
bioRxiv
Chronic pancreatitis patient-derived organoids reveal new paths to precision therapeutics. [Abstract]2025 Nov 14:2024.10.30.620903. PMID: 41279927 -
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Solvant et solubilité
DMSO : 50 mg/mL (127.39 mM; Need ultrasonic; Hygroscopic DMSO has a significant impact on the solubility of product, please use newly opened DMSO)
Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 1 year; -20°C, 6 months. When stored at -80°C, please use it within 1 year. When stored at -20°C, please use it within 6 months.
Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 1 year; -20°C, 6 months. When stored at -80°C, please use it within 1 year. When stored at -20°C, please use it within 6 months.
Concentration (start) × Volume (start) = Concentration (final) × Volume (final)
Select the appropriate dissolution method based on your experimental animal and administration route.
- For the following dissolution methods, please ensure to first prepare a clear stock solution using an In Vitro approach and then sequentially add co-solvents:
- To ensure reliable experimental results, the clarified stock solution can be appropriately stored based on storage conditions. As for the working solution for In Vivo experiments, it is recommended to prepare freshly and use it on the same day.
- The percentages shown for the solvents indicate their volumetric ratio in the final prepared solution. If precipitation or phase separation occurs during preparation, heat and/or sonication can be used to aid dissolution.
Add each solvent one by one: 10% DMSO 40% PEG300 5% Tween-80 45% Saline
Solubility: ≥ 2.5 mg/mL (6.37 mM); Clear solution
This protocol yields a clear solution of ≥ 2.5 mg/mL (saturation unknown).
Taking 1 mL working solution as an example, add 100 μL DMSO stock solution (25.0 mg/mL) to 400 μL PEG300, and mix evenly; then add 50 μL Tween-80 and mix evenly; then add 450 μL Saline to adjust the volume to 1 mL.
Preparation of Saline: Dissolve 0.9 g sodium chloride in ddH₂O and dilute to 100 mL to obtain a clear Saline solution.
Please enter the basic information of animal experiments:
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Recommended: Prepare an additional quantity of animals to account for potential losses during experiments.
Please enter your animal formula composition:
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%DMSO +
Recommended: Keep the proportion of DMSO in working solution below 2% if your animal is weak.
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%+
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+%Tween-80 + +
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%Saline +
The co-solvents required include: DMSO, . All of co-solvents are available by MedChemExpress (MCE). , Tween 80. All of co-solvents are available by MedChemExpress (MCE).
Working solution concentration: 0.22 mg/mL
Method for preparing stock solution: mg drug dissolved in μL DMSO. Stock solution concentration: mg/mL.
1. Take μL DMSO stock solution;
2. Add μL .
μL , mix evenly;
3. Then add μL Tween 80, mix evenly;
4. Then add μL
Please ensure that the stock solution in the first step is dissolved to a clear state, and add co-solvents in sequence. You can use ultrasonic heating (ultrasonic cleaner, recommended frequency 20-40 kHz), vortexing, etc. to assist dissolution.
Pureté et documentation
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Fiche technique (277 KB)
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SDS (393 KB)
- English - EN (393 KB)
- Français - FR (393 KB)
- Deutsch - DE (393 KB)
- Norwegian - NO (393 KB)
- Español - ES (393 KB)
- Swedish - SV (393 KB)
- Italian - IT (393 KB)
- Korean - KR (393 KB)
- Portuguese - PT (393 KB)
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Instruction de manipulation (2659 KB)
Références
[1]. Delaunay JL, et al. Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770). Hepatology. 2017 Feb;65(2):560-570 [Content Brief]
[2]. Mutyam V, et al. Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation. J Cyst Fibros. 2017 Jan;16(1):24-29 [Content Brief]
[3]. Hadida S, et al. Discovery of N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide (VX-770, ivacaftor), a potent and orally bioavailable CFTR potentiator. J Med Chem. 2014 Dec 11;57(23):9776-9 [Content Brief]
[4]. 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. [Content Brief]
Complete Stock Solution Preparation Table
Please refer to the solubility information to select the appropriate solvent. Once prepared, please aliquot and store the solution to prevent product inactivation from repeated freeze-thaw cycles.
Storage method and period of stock solution: -80°C, 1 year; -20°C, 6 months. When stored at -80°C, please use it within 1 year. When stored at -20°C, please use it within 6 months.
| Optional Solvent | Concentration Solvent Mass | 1 mg | 5 mg | 10 mg | 25 mg |
|---|---|---|---|---|---|
| DMSO | 1 mM | 2.5478 mL | 12.7392 mL | 25.4784 mL | 63.6959 mL |
| 5 mM | 0.5096 mL | 2.5478 mL | 5.0957 mL | 12.7392 mL | |
| 10 mM | 0.2548 mL | 1.2739 mL | 2.5478 mL | 6.3696 mL | |
| 15 mM | 0.1699 mL | 0.8493 mL | 1.6986 mL | 4.2464 mL | |
| 20 mM | 0.1274 mL | 0.6370 mL | 1.2739 mL | 3.1848 mL | |
| 25 mM | 0.1019 mL | 0.5096 mL | 1.0191 mL | 2.5478 mL | |
| 30 mM | 0.0849 mL | 0.4246 mL | 0.8493 mL | 2.1232 mL | |
| 40 mM | 0.0637 mL | 0.3185 mL | 0.6370 mL | 1.5924 mL | |
| 50 mM | 0.0510 mL | 0.2548 mL | 0.5096 mL | 1.2739 mL | |
| 60 mM | 0.0425 mL | 0.2123 mL | 0.4246 mL | 1.0616 mL | |
| 80 mM | 0.0318 mL | 0.1592 mL | 0.3185 mL | 0.7962 mL | |
| 100 mM | 0.0255 mL | 0.1274 mL | 0.2548 mL | 0.6370 mL |
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