| 货号 | 15145-1mg |
| 描述 | The cystic fibrosis (CF) gene encodes a cAMP-regulated chloride channel, the CF transmembrane conductance regulator (CFTR).1 A glycine-to-aspartic acid missense mutation at codon 551 (G551D) is a common mutation associated with a severe CF phenotype.1 Ivacaftor is an orally bioavailable CFTR potentiator that improves chloride transport. It increases the forskolin-induced CFTR-mediated epithelial current in cells expressing the G551D mutation by ~4-fold (EC50 = 100 nM) but has no effect on current in the absence of forskolin.2 Ivacaftor increases chloride secretion in cultured human CF bronchial epithelial cells carrying the G551D mutation on one allele and the common delta F508 processing mutation on the other allele.2 It binds CFTR directly and causes CFTR channel opening by an ATP-independent mechanism.3 It effectively improves sweat chloride in vivo while improving lung function when at least one G551D CFTR mutation is present.4 |
| 别名 | Kalydeco;VX 770; |
| 供应商 | Cayman |
| 应用文献 | |
| 1.Derand, R.,Bulteau-Pignoux, L. and Becq, F. The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory genistein binding site. The Journal of Biological Chemisty 277(39), 35999-36004 (2002). 2.Van Goor, F.,Hadida, S.,Grootenhuis, P.D., et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proceedings of the National Academy of Sciences of the United States of America 106(44), 18825-18830 (2009). 3.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 manner. The Journal of Biological Chemisty 287(44), 36639-36649 (2012). 4.Sloane, P.A. and Rowe, S.M. Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Curr.Opin.Pulm.Med. 16(6), 591-597 (2010). | |
| 运输条件 | Room temperature in continental US; may vary elsewhere |
| 存放说明 | -20 |
| 纯度 | ≥98% |
| 计算分子量 | 392.5 |
| 分子式 | C24H28N2O3 |
| CAS号 | 873054-44-5 |
| 稳定性 | ≥ 2 years |
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