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Journal of Cystic Fibrosis, ISSN 1569-1993, 09/2018, Volume 17, Issue 5, pp. 595 - 606
Current modulator therapies for some cystic fibrosis-causing CFTR mutants, including N1303K, have limited efficacy. We provide evidence here to support... 
High-throughput screen | Cystic fibrosis | N1303K | CFTR | Potentiator | CORRECTORS | ORGANOIDS | TRANSMEMBRANE CONDUCTANCE REGULATOR | IN-VITRO | RESCUE | RESPIRATORY SYSTEM | SYNERGY | VX-809 | CYSTIC-FIBROSIS | MUTATIONS | G551D | Care and treatment | Flavonoids | Flavones | Bioflavonoids | high-throughput screen | potentiator
Journal Article
Journal Article
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
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 11/2009, Volume 106, Issue 44, pp. 18825 - 18830
Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a protein kinase A... 
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 | epithelial sodium channel (ENaC) | ION-TRANSPORT | MULTIDISCIPLINARY SCIENCES | ENAC | IDENTIFICATION | TRANSMEMBRANE CONDUCTANCE REGULATOR | CHLORIDE-CHANNEL | FLUID SECRETION | cystic fibrosis transmembrane conductance regulator (CFTR) | SURFACE LIQUID VOLUME | DISEASE | MUTATIONS | epithelial ion transport | human bronchial epithelium (HBE) culture | CYSTIC-FIBROSIS GENE | 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 | Proteins | Cell culture | Membranes | Mutation | Gene expression | Kinases | Index Medicus | Biological Sciences
Journal Article
Journal Article
Journal of General Physiology, ISSN 0022-1295, 12/2017, Volume 149, Issue 12, pp. 1105 - 1118
Cystic fibrosis (CF) is a channelopathy caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which... 
CHLORIDE-CHANNEL | ATP HYDROLYSIS | IN-VITRO | DOMAIN | PHYSIOLOGY | SIDE-CHAIN | ION-CHANNEL | CYSTIC-FIBROSIS MUTATION | CL-CHANNEL | CONDUCTANCE-REGULATOR CFTR | BINDING | Cystic fibrosis | Ion channels | Research | Adenosine triphosphate | Binding | Phosphorylation | Channelopathy | Channel gating | Genes | Probability | Drug screening | Pyrazole | Resistance | Allosteric properties | Fibrosis | Reagents | Affinity | Mutation | Kinetics | Dimerization | Gating | ATP | Index Medicus | 514
Journal Article
Journal Article
Journal of Cystic Fibrosis, ISSN 1569-1993, 09/2019, Volume 18, Issue 5, pp. 693 - 699
Investigation of novel cystic fibrosis transmembrane conductance regulator (CFTR) potentiators, such as GLPG1837, for CF patients with gating mutations is... 
Cystic fibrosis | Ivacaftor | G551D | CFTR modulator | Gating mutation | GLPG1837 | Index Medicus
Journal Article
Bioorganic & Medicinal Chemistry Letters, ISSN 0960-894X, 2010, Volume 20, Issue 1, pp. 87 - 91
A developing therapy of cystic fibrosis caused by the ΔF508 mutation in CFTR employs correction of defective CFTR chloride channel gating by a ‘potentiator’... 
Potentiators | ΔF508-CFTR | Correctors | Hybrids | Cystic fibrosis transmembrane conductance regulator protein (CFTR) | Multi-ligand drug | TRANSMEMBRANE CONDUCTANCE REGULATOR | Delta F508-CFTR | CHEMISTRY, MEDICINAL | CHEMISTRY, ORGANIC | CFTR | SMALL-MOLECULE CORRECTORS | multi-ligand drug | hybrids | correctors | potentiators
Journal Article