| Identification | Back Directory | [Name]
Kalydeco hydrate | [CAS]
1134822-07-3 | [Synonyms]
VX770 hydrate
VX-770 hydrate
VX 770 hydrate
Kalydeco hydrate
Ivacaftor (hydrate)
Ivacaftor hydrate (VX-770)
Ivacaftor hydrate
(VX-770 hydrate
(Z)-N-(2,4-DI-TERT-BUTYL-5-HYDROXYPHENYL)-4-OXO-1,4-DIHYDROQUINOLINE-3-CARBIMIDIC ACID HYDRATE | [Molecular Formula]
C24H30N2O4 | [MOL File]
1134822-07-3.mol | [Molecular Weight]
410.51 |
| Hazard Information | Back Directory | [Description]
Ivacaftor hydrate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment. | [in vitro]
In Vitro: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]. | [in vivo]
In Vivo:Ivacaftor (1-200 mg/kg, p.o.) exhibits good oral bioavailability in rat[3]. | [References]
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.
[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.
[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):9776-95
[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. |
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SPIRO PHARMA
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www.spiropharma.com.cn |
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Musechem
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+1-800-259-7612 |
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www.musechem.com |
| Company Name: |
InvivoChem
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13549236410 |
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https://www.invivochem.cn/ |
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