Ivacaftor (VX-770)

Cas No.:	873054-44-5
Chemical Name:	VX770
Synonyms:	VX770;Ivacaftor (VX-770);N-[2,4-Bis(tert-butyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxo-3-quinolinecarboxamide;Ivacaftor;N-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide;VX-770;[14C]-Ivacaftor;N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide;N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxaraide;UNII-1Y740ILL1Z;VX770,VX 770,Ivacaftor
SMILES:	CC(C1=C(O)C=C(NC(C2=CNC3=CC=CC=C3C2=O)=O)C(C(C)(C)C)=C1)(C)C
Formula:	C24H28N2O3
M.Wt:	392.49072
Sotrage:	2 years -20°C Powder, 2 weeks 4°C in DMSO, 6 months -80°C in DMSO

Description:	Ivacaftor is a potent and orally bioavailable CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively.
In Vivo:	Ivacaftor (1-200 mg/kg, p.o.) exhibits good oral bioavailability in rat[3].
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].