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Article

Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.

Molecular pharmacology - 1 Aug 2018

Chin Stephanie, Hung Maurita, Won Amy, Wu Yu-Sheng, Ahmadi Saumel, Yang Donghe, Elmallah Salma, Toutah Krimo, Hamilton C Michael, Young Robert N, Viirre Russell D, Yip Christopher M, Bear Christine E

Abstract excerpt

Deletion of phenylalanine at position 508 (F508del) in cystic fibrosis transmembrane conductance regulator (CFTR) is the most common cystic fibrosis (CF)-causing mutation. Recently, ORKAMBI, a combination therapy that includes a corrector of the processing defect of F508del-CFTR (lumacaftor or VX-809) and a potentiator of channel activity (ivacaftor or VX-770), was approved for CF patients homozygous for this...

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PMID: 29903751
DOI: 10.1124/mol.118.112177

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CFTR

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Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.PMID 29903751Read ↗

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Semantic proximity Read ↗

Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.Semantic proximity Read ↗

Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.Semantic proximity Read ↗

PTI-801 (posenacaftor) shares a common mechanism with VX-445 (elexacaftor) to rescue p.Phe508del-CFTR.Semantic proximity Read ↗

Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.Semantic proximity Read ↗

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosisSemantic proximity Read ↗

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.Semantic proximity Read ↗

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent MannerSemantic proximity Read ↗

Characterization of the mechanism of action of RDR01752, a novel corrector of F508del-CFTR.Semantic proximity Read ↗

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.Semantic proximity Read ↗

Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis‐causing mutationSemantic proximity Read ↗

In Silico Evaluation of Lumacaftor for Cystic FibrosisSemantic proximity Read ↗

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