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Journal Article
American journal of respiratory and critical care medicine, ISSN 1073-449X, 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... 
PH | 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 | Index Medicus | Abridged Index Medicus | Original
Journal Article
Nature Cell Biology, ISSN 1465-7392, 09/2010, Volume 12, Issue 9, pp. 863 - 875
Accumulation of unwanted/misfolded proteins in aggregates has been observed in airways of patients with cystic fibrosis (CF), a life-threatening genetic... 
APOPTOSIS | UNFOLDED PROTEIN RESPONSE | BECLIN 1 | DOWN-REGULATION | ENDOPLASMIC-RETICULUM | MICE | GENE-TRANSFER | TISSUE TRANSGLUTAMINASE | NEURODEGENERATIVE DISEASES | CELL-DEATH | CELL BIOLOGY | Reactive Oxygen Species - metabolism | Respiratory Mucosa - drug effects | Sequestosome-1 Protein | Humans | Transglutaminases - genetics | Phosphatidylinositol 3-Kinases - antagonists & inhibitors | Autophagy - drug effects | Young Adult | Cystamine - therapeutic use | Inflammation - metabolism | Autophagy - genetics | Nasal Polyps - metabolism | Mice, Inbred CFTR | Respiratory Mucosa - cytology | Cystic Fibrosis - metabolism | Membrane Proteins - genetics | Mice, Transgenic | Protein Transport - genetics | Apoptosis Regulatory Proteins - metabolism | Reactive Oxygen Species - antagonists & inhibitors | Models, Biological | Cystic Fibrosis - genetics | Transglutaminases - metabolism | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Mice | GTP-Binding Proteins | Cystic Fibrosis - drug therapy | Protein Binding - physiology | Microtubule-Associated Proteins - metabolism | Phosphatidylinositol 3-Kinases - metabolism | Salicylates - pharmacology | Epithelial Sodium Channels - genetics | Apoptosis Regulatory Proteins - genetics | Adult | Cystamine - pharmacology | Membrane Proteins - metabolism | Acetylcysteine - therapeutic use | Beclin-1 | Cystic Fibrosis Transmembrane Conductance Regulator - antagonists & inhibitors | Cell Line | Small Ubiquitin-Related Modifier Proteins - metabolism | Heat-Shock Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Antioxidants - pharmacology | Mice, Inbred Strains | Nasal Polyps - drug therapy | Antioxidants - therapeutic use | Animals | Acetylcysteine - pharmacology | Adaptor Proteins, Signal Transducing - genetics | Respiratory Mucosa - metabolism | Adaptor Proteins, Signal Transducing - metabolism | Organometallic Compounds - pharmacology | Complications and side effects | Active oxygen | Gene mutations | Lung diseases | Physiological aspects | Cystic fibrosis | Genetic aspects | Research | Health aspects | Risk factors | Index Medicus
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 06/2013, Volume 123, Issue 6, pp. 2685 - 2693
Cystic fibrosis (CF) pigs develop disease with features remarkably similar to those in people with CF, including exocrine pancreatic destruction, focal biliary... 
TRANSMEMBRANE CONDUCTANCE REGULATOR | MEDICINE, RESEARCH & EXPERIMENTAL | PORCINE MODEL | MESSENGER-RNA | GENE | CELL-SPECIFIC EXPRESSION | NUCLEAR TRANSFER | PSEUDOMONAS-AERUGINOSA | EXACERBATIONS | AIRWAY EPITHELIA | MOUSE MODELS | Ileus - metabolism | Ileum - pathology | Humans | Cystic Fibrosis - pathology | Ileum - metabolism | Male | Meconium - metabolism | Lung - diagnostic imaging | Trachea - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - biosynthesis | Female | Ileus - pathology | Lung - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - deficiency | Infant, Newborn | Disease Models, Animal | Animals, Newborn | Promoter Regions, Genetic | Gene Expression | Lung - pathology | Animals, Genetically Modified | Cystic Fibrosis - metabolism | Pancreas - pathology | Rats | Pancreas - metabolism | Fatty Acid-Binding Proteins - genetics | Radiography | Trachea - pathology | Phenotype | Animals | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Sus scrofa | Care and treatment | Swine | Physiological aspects | Cystic fibrosis | Development and progression | Genetic aspects | Diagnosis | Research | Membrane proteins | Proteins | Hogs | Genotype & phenotype | Ostomy | Newborn babies | Pathogenesis | Cloning | Colleges & universities | Evacuations & rescues | Index Medicus | Abridged Index Medicus | Technical Advance
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 03/2016, Volume 126, Issue 3, pp. 879 - 891
Journal Article
Nature, ISSN 0028-0836, 2012, Volume 486, Issue 7405, pp. 109 - 113
Journal Article