Product Description
Ivacaftor (VX-770) is a potent, cell-permeable small molecule potentiator of cystic fibrosis transmembrane conductance regulator (CFTR). 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. Ivacaftor increases chloride secretion in cultured human CF bronchial epithelial cells carrying the G551D mutation on one allele and the common F508del processing mutation on the other allele. It binds CFTR directly and causes CFTR channel opening by an ATP-independent mechanism. It effectively improves sweat chloride in vivo while improving lung function when at least one G551D CFTR mutation is present.
Ivacaftor (VX-770) is a potent, cell-permeable small molecule potentiator of cystic fibrosis transmembrane conductance regulator (CFTR). 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. Ivacaftor increases chloride secretion in cultured human CF bronchial epithelial cells carrying the G551D mutation on one allele and the common F508del processing mutation on the other allele. It binds CFTR directly and causes CFTR channel opening by an ATP-independent mechanism. It effectively improves sweat chloride in vivo while improving lung function when at least one G551D CFTR mutation is present.
Biovision | 9582 | Ivacaftor DataSheet
Alternate Name/Synonyms: N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxo-3-quinolinecarboxamide; Cystic Fibrosis Transmembrane Conductance Regulator Potentiator; VX770; Kalydeco
Appearance: Off-white solid
Formulation: N/A
CAS Number: 873054-44-5
Structure Available?: Yes
Peptide sequence: N/A
Salt Form: No
Molecular Formula: C₂₄H₂₈N₂O₃
Molecular Weight: 392.49
Cell-Permeable?: Yes
Purity: ≥98% by HPLC
Solubilities: DMSO (>50 mg/ml)
Handling: Protect from air and light
Country of Origin: USA
Tag Line: A small molecule potentiator of cystic fibrosis transmembrane conductance regulator (CFTR).
MDL Number: MFCD17171361
PubChem CID: 16220172
SMILES: CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C
InChi: InChI=1S/C24H28N2O3/c1-23(2,3)16-11-17(24(4,5)6)20(27)12-19(16)26-22(29)15-13-25-18-10-8-7-9-14(18)21(15)28/h7-13,27H,1-6H3,(H,25,28)(H,26,29)
InChi Key: PURKAOJPTOLRMP-UHFFFAOYSA-N