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Journal Article
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
American journal of respiratory cell and molecular biology, ISSN 1044-1549, 11/2010, Volume 43, Issue 5, pp. 607 - 616
Journal Article
PloS one, ISSN 1932-6203, 2015, Volume 10, Issue 6, p. e0130313
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