Ivacaftor (Standard)

Ivacaftor (Standard) is the analytical standard of Ivacaftor. This product is intended for research and analytical applications. Ivacaftor (VX-770) is a potent and orally bioavailable CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively.

Product Specifications

CAS Number

[873054-44-5]

UNSPSC

12352100

Target

Autophagy; CFTR; Reference Standards

Related Pathways

Autophagy; Membrane Transporter/Ion Channel; Others

Field of Research

Endocrinology

Smiles

CC(C)(C1=C(C=C(C(C(C)(C)C)=C1)O)NC(C2=CNC3=C(C2=O)C=CC=C3)=O)C

Molecular Formula

C24H28N2O3

Molecular Weight

392.49

References & Citations

[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) :9776-9|[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.