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Ivacaftor benzenesulfonate

依伐卡托苯磺酸盐；VX-770 benzenesulfonate

目录号: PC11870 纯度: ≥98%

CFTR增强剂,Ivacaftor benzenesulfonate 是一种可口服的 CFTR 增强剂，常用于囊性纤维化的治疗。

CAS No. :1134822-09-5

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中文名称	Ivacaftor benzenesulfonate
英文名称	Ivacaftor benzenesulfonate
英文别名	Ivacaftor (benzenesulfonate);Kalydeco benzenesulfonate;VX 770 benzenesulfonate;VX-770 benzenesulfonate
Cas No.	1134822-09-5
分子式	C₃₀H₃₄N₂O₆S
分子量	550.67
包装储存	Store at -20°C
详情描述	CFTR增强剂,Ivacaftor benzenesulfonate 是一种可口服的 CFTR 增强剂，常用于囊性纤维化的治疗。

生物活性	Ivacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
性状	A solid
体外研究(In Vitro)	Ivacaftor (10 µM) increases the PC secretion activity by 3-fold for ABCB4-G535D, 13.7-fold for ABCB4-G536R, 6.7-fold for ABCB4-S1076C, 9.4-fold for ABCB4-S1176L, and 5.7-fold for ABCB4-G1178S. Ivacaftor corrects the functional defect of ABCB4 mutants[1]. Ivacaftor (10 μM) significantly increases CFTR activity in W1282X-expressing cells compared to R1162X CFTR cells[2]. Ivacaftor shows no significant activity against 160 targets tested including the GABAA benzodiazepine receptor. Ivacaftor increases the chloride secretion with an EC50 of 0.236 ± 0.200 μM, a 10-fold shift in potency compared to the F508del HBEs[3]. In recombinant cells, VX-770 increases CFTR channel open probability (Po) in both the F508del processing mutation and the G551D gating mutation. VX-770 increases forskolin-stimulated IT in temperature-corrected F508del-FRT cells by appr 6-fold with an EC50 of 25 nM[4].
体内研究(In Vivo)	Ivacaftor (1-200 mg/kg, p.o.) exhibits good oral bioavailability in rat[3].
运输条件	蓝冰冷藏运输
储存方式	Store at -20°C
结构分类	信号通路 >> 跨膜转运 >> CFTR 研究领域 >> 内分泌
参考文献	[1]. 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 [2]. 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 [3]. 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 [4]. 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.

溶解度数据	≥55.1 mg/mL in DMSO; insoluble in H2O; ≥13.86 mg/mL in EtOH with gentle warming

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[1]. 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
[2]. 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
[3]. 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
[4]. 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.

1：一般建议：溶解度为Medlife测试数据，可能与文献描述存在差异。这是由于生产工艺和批次不同产生的正常现象。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。不同批次产品溶解度各有差异，仅做参考，具体以实验方案为准。

2：储存条件：粉末-20°C一般情况可以保存3年，溶于溶剂-80°C一般情况可以保存1年。不同产品及不同批次产品可能存在差异，请细致阅读产品信息，并辅助参考相关文献描述。

Ivacaftor benzenesulfonate

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