Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis

American Journal of Respiratory and Critical Care Medicine

Volumn 190, Issue 2, 2014, Pages 175-184

  Rowe, Steven M ^a   Heltshe, Sonya L ^b,c   Gonska, Tanj ^d   Donaldson, Scott H ^e   Borowitz, Druc ^f   Gelfond, Danie ^f   Sagel, Scott D ^g   Khan, Ume ^c   Mayer Hamblett, Nicol ^b,c   Van Dalfsen, Jill M ^c   Joseloff, Elizabet ^h   Ramsey, Bonnie W ^b,c  

^a and Clinical Center   (United States)

^b UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^c SEATTLE CHILDREN S RESEARCH INSTITUTE   (United States)

^d HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

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

^f UNIVERSITY AT BUFFALO   (United States)

^g UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

^h Cystic Fibrosis Foundation Therapeutics   (United States)

Author keywords

CFTR modulator; Cystic fibrosis; Ivacaftor; PH; Pseudomonas aeruginosa

Indexed keywords

G551D PROTEIN; IVACAFTOR; PROTEIN; UNCLASSIFIED DRUG; AMINOPHENOL DERIVATIVE; BIOLOGICAL MARKER; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; N-(2,4-DI-TERT-BUTYL-5-HYDROXYPHENYL)-4-OXO-1,4-DIHYDROQUINOLINE-3-CARBOXAMIDE; QUINOLONE DERIVATIVE; RESPIRATORY TRACT AGENT;

ADOLESCENT; ADULT; ARTICLE; BODY MASS; CHILD; COHORT ANALYSIS; CYSTIC FIBROSIS; FEMALE; FORCED EXPIRATORY VOLUME; GASTROINTESTINAL PH; GENE MUTATION; HOSPITALIZATION; HUMAN; MAJOR CLINICAL STUDY; MALE; MICROBIOME; MUCOCILIARY CLEARANCE; MULTICENTER STUDY; PH; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA; RANDOMIZED CONTROLLED TRIAL (TOPIC); SPUTUM; CFTR MODULATOR; DRUG EFFECT; FOLLOW UP; GENETIC MARKER; GENETICS; ISOLATION AND PURIFICATION; LUNG; METABOLISM; MICROBIOLOGY; MICROFLORA; MUTATION; PATHOPHYSIOLOGY; PSEUDOMONAS INFECTION; SMALL INTESTINE; STATISTICS; SWEAT; TREATMENT OUTCOME; YOUNG ADULT;

CFTR MODULATOR; CYSTIC FIBROSIS; IVACAFTOR; PH; PSEUDOMONAS AERUGINOSA;

ADOLESCENT; ADULT; AMINOPHENOLS; BIOLOGICAL MARKERS; CHILD; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; FEMALE; FOLLOW-UP STUDIES; FORCED EXPIRATORY VOLUME; GENETIC MARKERS; HOSPITALIZATION; HUMANS; HYDROGEN-ION CONCENTRATION; INTESTINE, SMALL; LUNG; MALE; MICROBIOTA; MUCOCILIARY CLEARANCE; MUTATION; PSEUDOMONAS AERUGINOSA; PSEUDOMONAS INFECTIONS; QUINOLONES; RESPIRATORY SYSTEM AGENTS; SPUTUM; SWEAT; TREATMENT OUTCOME; YOUNG ADULT;

EID: 84906952377     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201404-0703OC     Document Type: Article

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