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Ivacaftor-d19
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
Ivacaftor-d19图片
包装:1mg
规格:98%
市场价:4430元
分子量:411.6

产品介绍
An internal standard for the quantification of ivacaftor
货号:ajcx22692
CAS:1413431-22-7
分子式:C24H9D19N2O3
分子量:411.6
溶解度:Acetonitrile:Methanol (1:1): soluble,DMSO: soluble
纯度:98%
存储:Store at -20°C
库存:现货

Background:

Ivacaftor-d19is intended for use as an internal standard for the quantification of ivacaftor by GC- or LC-MS. Ivacaftor is an orally bioavailable potentiator of the cystic fibrosis transmembrane conductance regulator (CFTR) that improves chloride transport.1It increases the forskolin-induced CFTR-mediated epithelial current in cells expressing the G551D missense mutation associated with severe cystic fibrosis by approximately 4-fold (EC50= 100 nM) but has no effect on current in the absence of forskolin.1Ivacaftor increases chloride secretion in cultured human cystic fibrosis bronchial epithelial cells carrying the G551D mutation on one allele and the common δF508 processing mutation on the other allele.1It binds CFTR directly and leads to CFTR channel openingviaan ATP-independent mechanism.2Formulations containing ivacaftor have been used in the treatment of cystic fibrosis in patients carrying one or more mutations in theCFTRgene.


1.Van Goor, F., Hadida, S., Grootenhuis, P.D., et al.Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770Proc. Natl. Acad. Sci. U.S.A.106(44)18825-18830(2009) 2.Eckford, P.D., Li, C., Ramjeesingh, M., et al.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent mannerJ. Biol. Chem.287(44)36639-36649(2012)