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Ivacaftor (VX-770)

现货
Catalog No.
A5047
CFTR增强剂
组合的产品项目
规格价格库存 数量
10mM (in 1mL DMSO)
¥ 800.00
现货
5mg
¥ 700.00
现货
25mg
¥ 2,500.00
现货
100mg
¥ 6,500.00
现货

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Background

Ivacaftor (VX-770) is a potent and orally bioavailable small molecule potentiator of cystic fibrosis transmembrane conductance regulator (CFTR). Studies in both G551D- and F508del-CFTR expressing cells have shown VX-700 combined with forskolin, but not VX-700 alone, has significantly increased CFTR-mediated Cl-secretion. The EC50 values of VX-700 in G551D- and F508del-CFTR are 100 nM and 25 nM, respectively [1].

VX-770 has been reported to reduce ENaC-mediated Na+ absorption and increase the amount of fluid on the apical surface in human CF bronchial epithelia (HBE) carrying G551D/F508del [1]. VX-770 combined with OAG has been shown to increase G551D-CFTR activity and OAG-dependent Ca2+ influx and in Chinese hamster ovary (CHO) cells [2].

References:
1. Van Goor F1, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J,Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P. 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.
2. Vachel L1, Norez C, Becq F, Vandebrouck C. Effect of VX-770 (ivacaftor) and OAG on Ca2+ influx and CFTR activity in G551D and F508del-CFTR expressing cells. J Cyst Fibros. 2013 Dec;12(6):584-91

文献引用

1. Phua YL, Chen KH, et al. "Loss of miR-17~92 results in dysregulation of Cftr in nephron progenitors." Am J Physiol Renal Physiol. 2019 May 1;316(5):F993-F1005. PMID:30838872

Chemical Properties

Physical AppearanceA solid
StorageStore at -20°C
M.Wt392.49
Cas No.873054-44-5
FormulaC24H28N2O3
Solubility≥19.6mg/mL in DMSO, ≥2.95 mg/mL in EtOH with ultrasonic, <2.53 mg/mL in H2O
Chemical NameN-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide
SDFDownload SDF
Canonical SMILESCC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C
运输条件试用装:蓝冰运输。 其他可选规格:常温运输或根据您的要求用蓝冰运输。
一般建议为了使其更好的溶解,请用37℃加热试管并在超声波水浴中震动片刻。不同厂家不同批次产品溶解度各有差异,仅做参考。若实验所需浓度过大至产品溶解极限,请添加助溶剂助溶或自行调整浓度。

试验操作

细胞实验 [1]:

细胞系

CHO-G551D细胞

制备方法

在DMSO中的溶解度大于10 mM。若配制更高浓度的溶液,一般步骤如下:请将试管置于37 °C加热10分钟和/或将其置于超声波浴中震荡一段时间。原液于-20 °C可放置数月。

反应条件

10 μM;0 ~ 8分钟

实验结果

在CHO-G551D细胞中,VX-770活化G551D-CFTR通道。此外,OAG + VX-770增加G551D-CFTR活性(58%)。

References:

[1]. Vachel L1, Norez C, Becq F, Vandebrouck C. Effect of VX-770 (ivacaftor) and OAG on Ca2+ influx and CFTR activity in G551D and F508del-CFTR expressing cells. J Cyst Fibros. 2013 Dec;12(6):584-91.

生物活性

描述 Ivacaftor (VX-770)是一种CFTR增强剂,靶向作用于G551D-CFTR和F508del-CFTR,EC50值分别为100 nM和25 nM。
靶点 G551D-CFTR F508del-CFTR        
IC50 100 nM 25 nM        

质量控制

相关生物数据

Ivacaftor
Ivacaftor inhibits mutant CFTR.

相关生物数据

Ivacaftor