Shanghai Nianxing Industrial Co., Ltd
China
Product Name: Ivacaftor hydrate
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ShangHai DC Chemicals Co.,Ltd
China
Product Name: Ivacaftor hydrate
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DC Chemicals Limited
China
Product Name: Ivacaftor hydrate
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Dayang Chem (Hangzhou) Co., Ltd.
China
Product Name: Kalydeco hydrate
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1134822-07-3

1134822-07-3 structure

1134822-07-3 structure

Name: Ivacaftor (hydrate)
Chemical Name: N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxamide.H2O
CAS Number: 1134822-07-3
Molecular Formula: C₂₄H₃₀N₂O₄
Molecular Weight: 410.50600
Catalog: Signaling Pathways Membrane Transporter/Ion Channel CFTR
Create Date: 2016-12-21 07:51:00
Modify Date: 2024-01-13 16:50:50
Ivacaftor hydrate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.

Name	N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxamide.H2O
Synonyms	Ivacaftor (hydrate)

Description	Ivacaftor hydrate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
Related Catalog	Signaling Pathways >> Membrane Transporter/Ion Channel >> CFTR Research Areas >> Endocrinology
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	Ivacaftor (1-200 mg/kg, p.o.) exhibits good oral bioavailability in rat[3].
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.

Molecular Formula	C₂₄H₃₀N₂O₄
Molecular Weight	410.50600
Exact Mass	410.22100
PSA	91.42000
LogP	5.08970
Storage condition	2-8℃