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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
American journal of physiology. Lung cellular and molecular physiology, ISSN 1522-1504, 2018, Volume 315, Issue 5, pp. L846 - L857
Journal Article
Journal Article
American journal of respiratory cell and molecular biology, ISSN 1044-1549, 11/2010, Volume 43, Issue 5, pp. 607 - 616
Journal Article
The Journal of clinical investigation, ISSN 0021-9738, 06/2013, Volume 123, Issue 6, pp. 2685 - 2693
.... We hypothesized that transgenic expression of porcine CF transmembrane conductance regulator (pCFTR) cDNA under control of the intestinal fatty acid-binding protein... 
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 | Technical Advance
Journal Article