Cystic fibrosis: Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression

Science Translational Medicine

Volumn 6, Issue 246, 2014, Pages

  Veit, Guido ^a   Avramescu, Radu G ^a   Perdomo, Doranda ^a   Phuan, Puay Wah ^b   Bagdany, Miklos ^a   Apaja, Pirjo M ^a   Borot, Florence ^a   Szollosi, Daniel ^c,d   Wu, Yu Sheng ^a   Finkbeiner, Walter E ^e   Hegedus, Tamas ^c,d   Verkman, Alan S ^b   Lukacs, Gergely L ^a  

^a MCGILL UNIVERSITY   (Canada)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c INSTITUTE OF EXPERIMENTAL MEDICINE   (Hungary)

^d SEMMELWEIS UNIVERSITY   (Hungary)

^e UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; LUMACAFTOR; AMINOPHENOL DERIVATIVE; CHLORIDE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508; QUINOLONE DERIVATIVE;

ARTICLE; CELL SURFACE; CONTROLLED STUDY; CYSTIC FIBROSIS; DRUG EFFICACY; ENDOPLASMIC RETICULUM; GENE MUTATION; HUMAN; HUMAN CELL; MEMBRANE STABILIZATION; NUCLEOTIDE BINDING DOMAIN 1; NUCLEOTIDE BINDING DOMAIN 2; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN STABILITY; RESPIRATORY EPITHELIUM; CASE REPORT; CELL COMPARTMENTALIZATION; CELL DENSITY; CELL FUNCTION; CELL IMMORTALIZATION; DOWN REGULATION; DRUG EXPOSURE; DRUG MECHANISM; ENDOCYTOSIS; MUTATIONAL ANALYSIS; PROTEIN TRANSPORT; BRONCHUS; CELL MEMBRANE; CHANNEL GATING; DRUG EFFECTS; EPITHELIUM CELL; GENE REPRESSION; GENETICS; METABOLISM; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; TIME FACTOR;

AMINOPHENOLS; BRONCHI; CELL MEMBRANE; CHLORIDES; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DOWN-REGULATION; ENDOCYTOSIS; EPITHELIAL CELLS; HUMANS; ION CHANNEL GATING; MUTATION; QUINOLONES; SUPPRESSION, GENETIC; TIME FACTORS;

EID: 84907211027     PISSN: 19466234     EISSN: 19466242     Source Type: Journal    
DOI: 10.1126/scitranslmed.3008889     Document Type: Article

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