Ivacaftor

• CAT Number: A000792
• CAS Number: 873054-44-5
• Molecular Formula: C24H28N2O3
• Molecular Weight: 392.50
• Purity: 98%
• Synonyms: VX-770
• Target: Autophagy
• Target Protein: P13569
• Solubility: >19.6mg/mL in DMSO
• Appearance: Solid
• Storage: Dry, dark and at 2 - 8 °C for six months or -20°C for two years.
• IC50: EC50: 100 nM(G551D-CFTR) and 25 nM(F508del-CFTR)
• IUPAC Name: N-(2,4-ditert-butyl-5-hydroxyphenyl)-4-oxo-1H-quinoline-3-carboxamide
• 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)
• InChIKey: PURKAOJPTOLRMP-UHFFFAOYSA-N
• SMILES: CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C

Ivacaftor（Cat No.:A000792）is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator used to treat cystic fibrosis (CF) in patients with specific CFTR gene mutations. It works by enhancing the activity of defective CFTR proteins, improving chloride transport across cell membranes and restoring the balance of salt and water in various tissues, particularly the lungs. Ivacaftor has shown significant benefits in improving lung function, reducing pulmonary exacerbations, and enhancing quality of life in CF patients. It is a personalized therapy targeting the underlying cause of CF for those with eligible mutations.