Ivacaftor-13C6

Ivacaftor-13C6 (VX-770-13C6) is 13C labeled Ivacaftor. 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

Product Name Alternative

VX-770-13C6

UNSPSC

12352005

Target

Autophagy; CFTR; Isotope-Labeled Compounds

Related Pathways

Autophagy; Membrane Transporter/Ion Channel; Others

Applications

Metabolism-protein/nucleotide metabolism

Field of Research

Endocrinology

Smiles

O=C(/C(C(C)=O)=C/NC)NC1=CC(O)=C(C(C)(C)C)C=C1C(C)(C)C.[13C]2=[13C][13C]=[13C][13C]=[13C]2

Molecular Formula

C2013C6H30N2O3

Molecular Weight

424.48

References & Citations

[1]Russak EM, et al. Impact of Deuterium Substitution on the Pharmacokinetics of Pharmaceuticals. Ann Pharmacother. 2019;53 (2) :211-216.|[2]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|[3]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|[4]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|[5]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.