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Ivacaftor benzenesulfonate

CFTR Potentiator CAS# 1134822-09-5

Ivacaftor benzenesulfonate

2D Structure

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Ivacaftor benzenesulfonate

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Chemical Properties of Ivacaftor benzenesulfonate

Cas No. 1134822-09-5 SDF Download SDF
PubChem ID 78357768 Appearance Powder
Formula C30H34N2O6S M.Wt 550.67
Type of Compound N/A Storage Desiccate at -20°C
Synonyms VX-770 benzenesulfonate
Solubility 25℃: DMSO
Chemical Name benzenesulfonic acid;N-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide
SMILES CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C.C1=CC=C(C=C1)S(=O)(=O)O
Standard InChIKey YLVYFBOFAFDIBR-UHFFFAOYSA-N
Standard InChI InChI=1S/C24H28N2O3.C6H6O3S/c1-23(2,3)16-11-17(24(4,5)6)20(27)12-19(16)26-22(29)15-13-25-18-10-8-7-9-14(18)21(15)28;7-10(8,9)6-4-2-1-3-5-6/h7-13,27H,1-6H3,(H,25,28)(H,26,29);1-5H,(H,7,8,9)
General tips For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.Stock solution can be stored below -20℃ for several months.
We recommend that you prepare and use the solution on the same day. However, if the test schedule requires, the stock solutions can be prepared in advance, and the stock solution must be sealed and stored below -20℃. In general, the stock solution can be kept for several months.
Before use, we recommend that you leave the vial at room temperature for at least an hour before opening it.
About Packaging 1. The packaging of the product may be reversed during transportation, cause the high purity compounds to adhere to the neck or cap of the vial.Take the vail out of its packaging and shake gently until the compounds fall to the bottom of the vial.
2. For liquid products, please centrifuge at 500xg to gather the liquid to the bottom of the vial.
3. Try to avoid loss or contamination during the experiment.
Shipping Condition Packaging according to customer requirements(5mg, 10mg, 20mg and more). Ship via FedEx, DHL, UPS, EMS or other couriers with RT, or blue ice upon request.

Biological Activity of Ivacaftor benzenesulfonate

DescriptionIvacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.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.

Ivacaftor benzenesulfonate Dilution Calculator

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Preparing Stock Solutions of Ivacaftor benzenesulfonate

1 mg 5 mg 10 mg 20 mg 25 mg
1 mM 1.816 mL 9.0798 mL 18.1597 mL 36.3194 mL 45.3992 mL
5 mM 0.3632 mL 1.816 mL 3.6319 mL 7.2639 mL 9.0798 mL
10 mM 0.1816 mL 0.908 mL 1.816 mL 3.6319 mL 4.5399 mL
50 mM 0.0363 mL 0.1816 mL 0.3632 mL 0.7264 mL 0.908 mL
100 mM 0.0182 mL 0.0908 mL 0.1816 mL 0.3632 mL 0.454 mL
* Note: If you are in the process of experiment, it's necessary to make the dilution ratios of the samples. The dilution data above is only for reference. Normally, it's can get a better solubility within lower of Concentrations.

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Background on Ivacaftor benzenesulfonate

Description: IC50 Value: 25 nM (F508del-CFTR);100 nM (G551D-CFTR) [1] Ivacaftor (VX-770) is a CFTR Potentiator approved for patients with the G551D mutation of cystic fibrosis, which accounts for 4-5% cases of cystic fibrosis. in vitro: In recombinant cells VX-770 increased CFTR channel open probability (P(o)) in both the F508del processing mutation and the G551D gating mutation. VX-770 also increased Cl(-) secretion in cultured human CF bronchial epithelia (HBE) carrying the G551D gating mutation on one allele and the F508del processing mutation on the other allele by approximately 10-fold, to approximately 50% of that observed in HBE isolated from individuals without CF [1]. in vivo: At day 28, in the group of subjects who received 150 mg of VX-770, the median change in the nasal potential difference (in response to the administration of a chloride-free isoproterenol solution) from baseline was -3.5 mV (range, -8.3 to 0.5; P=0.02 for the within-subject comparison, P=0.13 vs. placebo), and the median change in the level of sweat chloride was -59.5 mmol per liter (range, -66.0 to -19.0; P=0.008 within-subject, P=0.02 vs. placebo) [2]. Toxicity: Six severe adverse events occurred in two subjects (diffuse macular rash in one subject and five incidents of elevated blood and urine glucose levels in one subject with diabetes). All severe adverse events resolved without the discontinuation of VX-770 [2]. Clinical trial: Roll-Over Study of Ivacaftor in Cystic Fibrosis Pediatric Subjects With a CFTR Gating Mutation. Phase 3

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Ivacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.

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