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 Manner

The cystic fibrosis transmembrane conductance regulator (CFTR) acts as a channel on the apical membrane of epithelia. Disease-causing mutations in the cystic fibrosis gene can lead to CFTR protein misfolding as in the case of the F508del mutation and/or channel dysfunction. Recently, a small molecul...

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Veröffentlicht in:	The Journal of biological chemistry 2012-10, Vol.287 (44), p.36639-36649
Hauptverfasser:	Eckford, Paul D.W., Li, Canhui, Ramjeesingh, Mohabir, Bear, Christine E.
Format:	Artikel
Sprache:	eng
Schlagworte:	ABC Transporter Adenosine Triphosphate - chemistry Aminophenols - chemistry Animals ATP Caprylates - chemistry Cell Line CFTR Chloride Channels Chromatography, Affinity Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator - chemistry Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - isolation & purification Detergents - chemistry Fluorocarbons - chemistry Humans Ion Channel Gating - drug effects Ion-sensitive Electrodes Liposomes Membrane Biology Membrane Proteins Mutant Proteins - chemistry Mutation, Missense Phosphorylation Protein Binding Protein Conformation - drug effects Protein Phosphorylation Protein Processing, Post-Translational Protein Purification Quinolones - chemistry Sequence Deletion Signal Transduction - drug effects Spodoptera
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