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American journal of respiratory and critical care medicine, ISSN 1535-4970, 2014, Volume 190, Issue 2, pp. 175 - 184
Rationale: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients with CF age 6 and older with the G551D mutation... 
Cystic fibrosis | Pseudomonas aeruginosa | Ivacaftor | CFTR modulator | LUNG-FUNCTION | ivacaftor | MUCIN SECRETION | ADRENERGIC SWEAT SECRETION | MUCOCILIARY CLEARANCE | INTESTINAL PH | CHILDREN | RESPIRATORY SYSTEM | cystic fibrosis | PSEUDOMONAS-AERUGINOSA | INFLAMMATION | DISEASE | QUESTIONNAIRE | CRITICAL CARE MEDICINE | Sputum - microbiology | Lung - microbiology | Pseudomonas aeruginosa - isolation & purification | Follow-Up Studies | Forced Expiratory Volume - drug effects | Sweat - drug effects | Humans | Microbiota - drug effects | Respiratory System Agents - therapeutic use | Hospitalization - statistics & numerical data | Male | Aminophenols - therapeutic use | Quinolones - pharmacology | Aminophenols - pharmacology | Mucociliary Clearance - drug effects | Pseudomonas Infections - prevention & control | Young Adult | Hydrogen-Ion Concentration - drug effects | Quinolones - therapeutic use | Adult | Female | Lung - metabolism | Child | Biomarkers - metabolism | Respiratory System Agents - pharmacology | Cystic Fibrosis - metabolism | Cystic Fibrosis - microbiology | Pseudomonas Infections - complications | Pseudomonas Infections - diagnosis | Treatment Outcome | Lung - physiopathology | Genetic Markers | Sputum - metabolism | Cystic Fibrosis - genetics | Intestine, Small - drug effects | Lung - drug effects | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Intestine, Small - metabolism | Mutation | Cystic Fibrosis - drug therapy | Sweat - metabolism | Original
Journal Article
The New England journal of medicine, ISSN 1533-4406, 2011, Volume 365, Issue 18, pp. 1663 - 1672
Journal Article
The New England journal of medicine, ISSN 1533-4406, 2017, Volume 377, Issue 21, pp. 2024 - 2035
Journal Article
American journal of respiratory cell and molecular biology, ISSN 1044-1549, 01/2017, Volume 56, Issue 1, pp. 99 - 108
Acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction may contribute to chronic obstructive pulmonary disease pathogenesis and is a potential therapeutic target... 
Cystic fibrosis transmembrane conductance regulator potentiator | Ivacaftor | Optical coherence tomography | Cigarette smoke | Mucociliary transport | ACTIVATION | BRONCHIAL EPITHELIAL-CELLS | optical coherence tomography | BIOCHEMISTRY & MOLECULAR BIOLOGY | cystic fibrosis transmembrane conductance regulator potentiator | ivacaftor | cigarette smoke | FUNCTION IN-VITRO | mucociliary transport | CELL BIOLOGY | AIRWAY | RESPIRATORY SYSTEM | SECRETION | DYSFUNCTION | MUTATIONS | EXPRESSION | RESIDUES | CFTR | Cystic Fibrosis Transmembrane Conductance Regulator - antagonists & inhibitors | Smoking - adverse effects | Acrolein - pharmacology | Amino Acid Sequence | Epithelial Cells - metabolism | Tomography, Optical Coherence | Epithelial Cells - drug effects | Humans | Cells, Cultured | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Quinolones - pharmacology | Cystic Fibrosis Transmembrane Conductance Regulator - chemistry | Aminophenols - pharmacology | Cilia - metabolism | Mucociliary Clearance - drug effects | Trachea - pathology | Mucous Membrane - pathology | Cilia - drug effects | Bronchi - pathology | Ion Channel Gating - drug effects | Proteins | Studies | Genotype & phenotype | Tomography | Cystic fibrosis | Software | Chronic obstructive pulmonary disease | Bronchitis | Kinases | Cigarettes
Journal Article
American journal of respiratory cell and molecular biology, ISSN 1044-1549, 11/2010, Volume 43, Issue 5, pp. 607 - 616
Journal Article
Journal Article
The New England journal of medicine, ISSN 1533-4406, 2017, Volume 377, Issue 21, pp. 2013 - 2023
Journal Article
American journal of respiratory cell and molecular biology, ISSN 1535-4989, 2014, Volume 50, Issue 4, pp. 805 - 816
.... Using a dual-luciferase reporter system containing the four most prevalent CF transmembrane conductance regulator (CFTR... 
Aminoglycosides | Ivacaftor | Cystic fibrosis transmembrane conductance regulator | Nonsense mutations | Translational readthrough | ANTIBIOTICS | translational readthrough | BIOCHEMISTRY & MOLECULAR BIOLOGY | GENETIC-DISEASES | ivacaftor | GENTAMICIN | CELL BIOLOGY | IN-VITRO | PREMATURE STOP MUTATIONS | GLYCOSAMINOGLYCAN ACCUMULATION | RESPIRATORY SYSTEM | PTC124 TREATMENT | TERMINATION CODONS | cystic fibrosis transmembrane conductance regulator | nonsense mutations | aminoglycosides | DUCHENNE MUSCULAR-DYSTROPHY | CF MOUSE MODEL | Luciferases - metabolism | Aminophenols - pharmacokinetics | Humans | Rats, Inbred F344 | Quinolones - pharmacology | Aminophenols - pharmacology | Luciferases - genetics | Cystic Fibrosis Transmembrane Conductance Regulator - drug effects | Transfection | Biological Transport | Time Factors | Quinolones - pharmacokinetics | Chlorides - metabolism | Organ of Corti - pathology | Genes, Reporter | Disease Models, Animal | Cell Line | Mice, Inbred CFTR | Aminoglycosides - pharmacokinetics | Cystic Fibrosis - metabolism | Codon, Nonsense - drug effects | Rats | Mice, Transgenic | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Drug Synergism | Animals | Aminoglycosides - toxicity | Cystic Fibrosis - genetics | Organ of Corti - drug effects | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Mice | Aminoglycosides - pharmacology | Aminoglycosides - chemical synthesis | Cystic Fibrosis - drug therapy | Proteins | Studies | Cell culture | Genetic disorders | Antibiotics | Toxicity | Rodents | Cystic fibrosis | Mutation | Reproductive system | Muscular dystrophy | Original Research
Journal Article