Cystic fibrosis: Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

Science Translational Medicine

Volumn 6, Issue 246, 2014, Pages

  Cholon, Deborah M ^a   Quinney, Nancy L ^a   Fulcher, M Leslie ^a   Esther Jr , Charles R ^a   Das, Jhuma ^a   Dokholyan, Nikolay V ^a   Randell, Scott H ^a   Boucher, Richard C ^a   Gentzsch, Martina ^a  

^a UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL AFFECTING AGENT; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL SODIUM CHANNEL; FORSKOLIN; IVACAFTOR; LUMACAFTOR; UNCLASSIFIED DRUG; URIDINE TRIPHOSPHATE; VX 661; VX 662; AMINOPHENOL DERIVATIVE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508; MUTANT PROTEIN; QUINOLONE DERIVATIVE;

AREA UNDER THE CURVE; ARTICLE; CONFORMATIONAL TRANSITION; CONTROLLED STUDY; CYSTIC FIBROSIS; DOSE RESPONSE; DRUG MECHANISM; G551D GENE; GENE MUTATION; HUMAN; HUMAN CELL; IN VITRO STUDY; NUCLEOTIDE BINDING SITE; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN CONFORMATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN LOCALIZATION; PROTEIN STABILITY; PROTEIN SYNTHESIS; THERMOSTABILITY; TREATMENT DURATION; BRONCHUS; CHEMICAL STRUCTURE; DRUG EFFECTS; EPITHELIUM CELL; GENETICS; METABOLISM; PATHOLOGY;

AMINOPHENOLS; BRONCHI; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DOSE-RESPONSE RELATIONSHIP, DRUG; EPITHELIAL CELLS; HUMANS; MODELS, MOLECULAR; MUTANT PROTEINS; PROTEIN STABILITY; QUINOLONES;

EID: 84907223425     PISSN: 19466234     EISSN: 19466242     Source Type: Journal    
DOI: 10.1126/scitranslmed.3008680     Document Type: Article

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