Ivacaftor (hydrate)
Names
Biological Activity
[Description]:
Ivacaftor hydrate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
[Related Catalog]:
[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]
[Related Small Molecules]
Chemical & Physical Properties
[ Molecular Formula ]:
C24H30N2O4
[ Molecular Weight ]:
410.50600
[ Exact Mass ]:
410.22100
[ PSA ]:
91.42000
[ LogP ]:
5.08970
[ Storage condition ]:
2-8℃