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cystic fibrosis (418) 418
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Proceedings of the National Academy of Sciences - PNAS, ISSN 1091-6490, 10/2009, Volume 106, Issue 44, pp. 18825 - 18830
.... Here we describe the in vitro pharmacology of VX-770, an orally bioavailable CFTR potentiator in clinical development for the treatment of CF... 
3T3 cells | Lungs | Secretion | Epithelial cells | Lung diseases | Bronchi | Cystic fibrosis | Pharmacology | Genetic mutation | Cilia | Epithelial sodium channel (ENaC) | Cystic fibrosis transmembrane conductance regulator (CFTR) | Epithelial ion transport | Human bronchial epithelium (HBE) culture | Science & Technology - Other Topics | Multidisciplinary Sciences | Science & Technology | NIH 3T3 Cells | Epithelial Cells - metabolism | Aminophenols - chemistry | Cystic Fibrosis - physiopathology | Epithelial Cells - drug effects | Humans | Quinolones - pharmacology | Epithelial Sodium Channels - metabolism | Sodium - metabolism | Aminophenols - pharmacology | Quinolines - pharmacology | Amino Acid Substitution - drug effects | Quinolones - chemistry | Chlorides - metabolism | Cilia - drug effects | Bronchi - pathology | Quinolines - chemistry | Cells, Cultured | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Mutation - genetics | Cilia - metabolism | Drug Synergism | Animals | Absorption - drug effects | Mice | Ion Channel Gating - drug effects | Care and treatment | Chemical properties | Protein kinases | Health aspects | Index Medicus | Biological Sciences | epithelial sodium channel (ENaC) | cystic fibrosis transmembrane conductance regulator (CFTR) | epithelial ion transport | human bronchial epithelium (HBE) culture
Journal Article
Journal of cystic fibrosis, ISSN 1569-1993, 09/2019, Volume 18, Issue 5, pp. 693 - 699
...) potentiators, such as GLPG1837, for CF patients with gating mutations is challenging as trials require patients to withhold ivacaftor, the current standard of care... 
Pulmonary/Respiratory | Cystic fibrosis | Ivacaftor | G551D | CFTR modulator | Gating mutation | GLPG1837 | Life Sciences & Biomedicine | Respiratory System | Science & Technology | Index Medicus
Journal Article
American journal of respiratory and critical care medicine, ISSN 1535-4970, 07/2014, 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 | Respiratory System | Life Sciences & Biomedicine | Critical Care Medicine | General & Internal Medicine | Science & Technology | 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 | Index Medicus | Abridged Index Medicus | ivacaftor | Original | cystic fibrosis
Journal Article
Autophagy, ISSN 1554-8627, 10/2014, Volume 8, Issue 11, pp. 1657 - 1672
Channel activators (potentiators) of cystic fibrosis (CF) transmembrane conductance regulator (CFTR... 
cystamine | CFTR potentiators | autophagy | cystic fibrosis | therapy | Binding | Proteins | Landes | Calcium | Bioscience | Biology | Cell | Cycle | Cancer | Organogenesis | Therapy | Cystic fibrosis | Autophagy | Cystamine | Life Sciences & Biomedicine | Science & Technology | Cell Biology | Inflammation - pathology | Epithelial Cells - metabolism | Sequestosome-1 Protein | Epithelial Cells - drug effects | Humans | Cystic Fibrosis - pathology | Male | Nasal Mucosa - pathology | Molecular Targeted Therapy | Autophagy - drug effects | Salicylates - pharmacology | Cystamine - therapeutic use | Organometallic Compounds - therapeutic use | Cystamine - pharmacology | Female | Cell Membrane - metabolism | Membrane Proteins - metabolism | Child | Cell Membrane - drug effects | Nasal Mucosa - drug effects | Beclin-1 | Epithelium - drug effects | Epithelium - pathology | Lung - pathology | Nasal Polyps - pathology | Epithelium - metabolism | Heat-Shock Proteins - metabolism | Epithelial Cells - pathology | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Antioxidants - pharmacology | Salicylates - therapeutic use | Apoptosis Regulatory Proteins - metabolism | Animals | Lung - drug effects | Adolescent | Genistein - pharmacology | Lipopolysaccharides - pharmacology | Mice | Adaptor Proteins, Signal Transducing - metabolism | Cystic Fibrosis - drug therapy | Organometallic Compounds - pharmacology | Index Medicus | Translational Research Paper
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Journal Article