Ivacaftor hydrate
Based on 29 publication(s) in Google Scholar
Ivacaftor hydrate (VX-770 hydrate) is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
For research use only. We do not sell to patients.
- CAS No.: 1134822-07-3
- Formula: C24H30N2O4
- Molecular Weight:410.51
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Storage:
Please store the product under the recommended conditions in the Certificate of Analysis.
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
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Biological Activity
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Chemical Information
- Purity & Documentation
- References
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Help & FAQs
Publications Citing Use of MedChemExpress (MCE) Ivacaftor hydrate
More- Acta Pharmacol Sin. 2025 Apr;46(4):1045-1057. [Abstract]
- JCI Insight. 2026 Mar 23;11(6):e198846. [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 Cyst Fibros. 2026 Jun 16:S1569-1993(26)01643-7. [Abstract]
- Am J Physiol Cell Physiol. 2025 Mar 1;328(3):C856-C871. [Abstract]
- Am J Respir Cell Mol Biol. 2025 Mar;72(3):308-319. [Abstract]
- Front Cell Dev Biol. 2021 May 11:9:678209. [Abstract]
- J Mol Struct. 2025 Nov 10;1353:144673.
- Sci Rep. 2025 Apr 21;15(1):13767. [Abstract]
- PNAS Nexus. 2025 Jul 4;4(7):pgaf211. [Abstract]
- Hum Gene Ther. 2024 Sep;35(17-18):695-709. [Abstract]
- Biomedicines. 2025 Jan 1;13(1):82. [Abstract]
- Ther Adv Med Oncol. 2026 Jan 2:18:17588359251409010. [Abstract]
- Microbiol Spectr. 2025 Sep 2;13(9):e0227524. [Abstract]
- J Biol Chem. 2024 Nov;300(11):107873. [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.
- Biomed Pharmacother. 2023 Oct:166:115399. [Abstract]
- 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
Biological Activity
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. 1134822-07-3
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Molecular Weight 410.51
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Formula C24H30N2O4
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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.O
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Synonyms
VX-770 hydrate
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Shipping
Room temperature in continental US; may vary elsewhere.
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Storage
Please store the product under the recommended conditions in the Certificate of Analysis.
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 -
JCI Insight
Targeting PI3Kγ anchoring enhances CFTR membrane localization and modulator efficacy via PKD1. [Abstract]2026 Mar 23;11(6):e198846. PMID: 41869727 -
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 -
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 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 -
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 -
Front Cell Dev Biol
2021 May 11:9:678209. PMID: 34046412
Ivacaftor hydrate 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 hydrate 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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Sci Rep
CFTR acts as a potential therapeutic target for attention deficit-hyperactivity disorder. [Abstract]2025 Apr 21;15(1):13767. PMID: 40258939 -
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 -
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 -
Biomedicines
2025 Jan 1;13(1):82. PMID: 39857666 -
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 -
Microbiol Spectr
Condition-dependent effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) on Aspergillus fumigatus growth. [Abstract]2025 Sep 2;13(9):e0227524. PMID: 40736245 -
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 -
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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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 hydrate 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 hydrate 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 hydrate 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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Purity & Documentation
References
[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]
Calculators
Concentration (start) × Volume (start) = Concentration (final) × Volume (final)
Ivacaftor hydrate Related Classifications
HY-13017B Related Classifications
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