Ivacaftor benzenesulfonate

CAS No.	1134822-09-5	Cat. No.	BCP19043
Name	Ivacaftor benzenesulfonate
Synonyms	VX-770 benzenesulfonate;VX770 benzenesulfonate;VX 770 benzenesulfonate;
Formula	C30H34N2O6S	M. Wt	550.67

Description	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 . 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) . Toxicity: Six severe adverse events occurred in two subjects (diffuse macular ra
Pathways	Ion Channel/Membrane Transporter
Targets	CFTR

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