Product Description: Ivacaftor-d4 (VX-770-d4) is the deuterium labeled-Ivacaftor (HY-13017). Ivacaftor is a potent and orally active CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively[1].
Applications: Metabolism-protein/nucleotide metabolism
Formula: C24H24D4N2O3
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):9776-9/[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.
Molecular Weight: 396.52
Compound Purity: 96.0
Research Area: Endocrinology
Solubility: 10 mM in DMSO/DMSO : 50mg/mL (ultrasonic)
Target: CFTR
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