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American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 01/2017, Volume 195
Rationale: Mutations that compromise protein levels, membrane expression or activity of the Cystic Fibrosis Transmembrane Conductance Regulator Channel (CFTR)... 
Bacterial infections | Cystic fibrosis | Infections | Mutation | Phosphatase
Journal Article
Journal Article
Nature Medicine, ISSN 1078-8956, 2013, Volume 19, Issue 7, pp. 939 - 945
Journal Article
Science, ISSN 0036-8075, 9/2008, Volume 321, Issue 5897, pp. 1837 - 1841
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 | Technical Advance
Journal Article
Free Radical Biology and Medicine, ISSN 0891-5849, 2001, Volume 30, Issue 12, pp. 1440 - 1461
Though the cause of cystic fibrosis (CF) pathology is understood to be the mutation of the CFTR protein, it has been difficult to trace the exact mechanisms by... 
Free radicals | NFκB | Reduced glutathione (GSH) | Lipid peroxidation | Cystic fibrosis | Inflammation | Antioxidant | CFTR | Nitric oxide (NO) | BRONCHOALVEOLAR LAVAGE FLUID | nitric oxide (NO) | antioxidant | free radicals | SECRETORY LEUKOPROTEASE INHIBITOR | NITRIC-OXIDE SYNTHASE | BIOCHEMISTRY & MOLECULAR BIOLOGY | LOWER RESPIRATORY-TRACT | MULTIDRUG-RESISTANCE PROTEIN | lipid peroxidation | reduced glutathione (GSH) | POLYMORPHONUCLEAR LEUKOCYTE FUNCTION | TUMOR-NECROSIS-FACTOR | inflammation | cystic fibrosis | ENDOCRINOLOGY & METABOLISM | INTERLEUKIN-2 RECEPTOR CONCENTRATIONS | EPITHELIAL LINING FLUID | NF-KAPPA-B | NF kappa B | Tumor Necrosis Factor-alpha - metabolism | Oxidative Stress | Glutathione - metabolism | Antioxidants - metabolism | Humans | Cystic Fibrosis - pathology | Cystic Fibrosis Transmembrane Conductance Regulator - physiology | NF-kappa B - metabolism | Infection - etiology | Contraindications | Cystic Fibrosis - immunology | Biological Transport | Mucus - metabolism | Lung - metabolism | Glutathione - deficiency | Cystic Fibrosis Transmembrane Conductance Regulator - deficiency | Drug Administration Routes | Endopeptidases - metabolism | Glutathione - therapeutic use | Lung - pathology | Disease Susceptibility | Cytokines - metabolism | Oxidation-Reduction | Cystic Fibrosis - metabolism | Gene Expression Regulation | Protease Inhibitors - metabolism | Glutathione - administration & dosage | Immune System - physiopathology | Models, Biological | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Nitric Oxide - metabolism | Cystic Fibrosis - drug therapy | Apoptosis
Journal Article