Two small molecules restore stability to a subpopulation of the cystic fibrosis transmembrane conductance regulator with the predominant disease-causing mutation

Journal of Biological Chemistry

Volumn 292, Issue 9, 2017, Pages 3706-3719

  Xin, Meng ^a   Yiting, Wang ^a   Xiaomeng, Wang ^a   Wrennall, Joe A ^b   Rimington, Tracy L ^a   Li, Hongyu ^b   Cai, Zhiwei ^b   Ford, Robert C ^a   Sheppard, David N ^b  

^a UNIVERSITY OF MANCHESTER   (United Kingdom)

^b UNIVERSITY OF BRISTOL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CELL MEMBRANES; MOLECULES; PLASMA STABILITY; PURIFICATION; RESTORATION; TEMPERATURE;

CHANNEL ACTIVITY; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATORS; LOW TEMPERATURES; SINGLE CHANNEL RECORDING; SINGLE CHANNELS; SMALL MOLECULES; TEMPORAL STABILITY;

CHLORINE COMPOUNDS;

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; LUMACAFTOR; 1,3 BENZODIOXOLE DERIVATIVE; AMINOPHENOL DERIVATIVE; AMINOPYRIDINE DERIVATIVE; CFTR PROTEIN, HUMAN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508; QUINOLONE DERIVATIVE;

ARTICLE; CELL SUBPOPULATION; CONTROLLED STUDY; ENZYME INACTIVATION; ENZYME INHIBITION; HUMAN; HUMAN CELL; INCUBATION TIME; LIMIT OF QUANTITATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN PURIFICATION; PROTEIN STABILITY; THERMOSTABILITY; ANIMAL; CELL FREE SYSTEM; CELL LINE; CELL MEMBRANE; CHROMATOGRAPHY; CYSTIC FIBROSIS; GENETICS; HAMSTER; HEAT; METABOLISM; MUTATION; PATCH CLAMP TECHNIQUE; PROTEIN DENATURATION;

AMINOPHENOLS; AMINOPYRIDINES; ANIMALS; BENZODIOXOLES; CELL LINE; CELL MEMBRANE; CELL-FREE SYSTEM; CHROMATOGRAPHY; CRICETINAE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HOT TEMPERATURE; HUMANS; MUTATION; PATCH-CLAMP TECHNIQUES; PROTEIN DENATURATION; QUINOLONES;

EID: 85014681640     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M116.751537     Document Type: Article

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