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Ivacaftor (benzenesulfonate)

Ivacaftor (benzenesulfonate)

品 牌 MCE
C A S 号 1134822-09-5
货 号 HY-13017A
规 格 5mg
价 格(元) 744.00
咨询优惠价
货号 规格 价格
HY-13017A 5mg 744.00
HY-13017A 10mg 1348.00
HY-13017A 50mg 4650.00
HY-13017A 100mg 6510.00

产品说明


Ivacaftor (VX-770)苯磺酸盐是CFTR的增强剂,对G551D-CFTR和F508del-CFTR的EC50分别为100 nM和25 nM。



Ivacaftor benzenesulfonate的生物活性


Ivacaftor benzenesulfonate (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.
IC50 Value: 25 nM (F508del-CFTR);100 nM (G551D-CFTR) [1]
Target: F508del-CFTR/G551D-CFTR
in vitro: In recombinant cells VX-770 increased CFTR channel open probability (P(o)) in both the F508del processing mutation and the G551D gating mutation. VX-770 also increased Cl(-) secretion in cultured human CF bronchial epithelia (HBE) carrying the G551D gating mutation on one allele and the F508del processing mutation on the other allele by approximately 10-fold, to approximately 50% of that observed in HBE isolated from individuals without CF [1].
in vivo: At day 28, in the group of subjects who received 150 mg of VX-770, the median change in the nasal potential difference (in response to the administration of a chloride-free isoproterenol solution) from baseline was -3.5 mV (range, -8.3 to 0.5; P=0.02 for the within-subject comparison, P=0.13 vs. placebo), and the median change in the level of sweat chloride was -59.5 mmol per liter (range, -66.0 to -19.0; P=0.008 within-subject, P=0.02 vs. placebo) [2].
Toxicity: Six severe adverse events occurred in two subjects (diffuse macular rash in one subject and five incidents of elevated blood and urine glucose levels in one subject with diabetes). All severe adverse events resolved without the discontinuation of VX-770 [2].
Clinical trial: Roll-Over Study of Ivacaftor in Cystic Fibrosis Pediatric Subjects With a CFTR Gating Mutation. Phase 3

化学信息


分子量 550.67 储存条件 参考CoA中推荐的条件进行储存。
分子式 C₃₀H₃₄N₂O₆S
CAS号 1134822-09-5
溶剂/溶解度 10 mM in DMSO

相关文献


[1]. 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.
[2]. Accurso FJ, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010 Nov 18;363(21):1991-2003.