G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations

G551D is a major disease-associated gating mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP- and phosphorylation-dependent chloride channel. G551D causes severe cystic fibrosis (CF) disease by disrupting ATP-dependent channel opening; however, whether G551D...

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Veröffentlicht in:	American journal of physiology. Lung cellular and molecular physiology 2020-11, Vol.319 (5), p.L770-L785
Hauptverfasser:	Wang, Wei, Fu, Lianwu, Liu, Zhiyong, Wen, Hui, Rab, Andras, Hong, Jeong S, Kirk, Kevin L, Rowe, Steven M
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenosine triphosphate Adenosine Triphosphate - metabolism Animals Binding Channel gating Channel opening Charge reversal Chloride Channels - drug effects Chloride Channels - genetics Chloride Channels - metabolism Chloride ions Cyclic AMP-Dependent Protein Kinases - metabolism Cystic fibrosis Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Humans Ion Channel Gating - drug effects Ion Channel Gating - genetics Ion channels Kinases Mutation Mutation - drug effects Mutation - genetics Nucleotides Phosphorylation Sensitivity Signal Transduction - drug effects
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