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Science, ISSN 0036-8075, 06/2019, Volume 364, Issue 6446, p. 1147
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 07/2015, Volume 373, Issue 3, pp. 220 - 231
Journal Article
Journal of Cystic Fibrosis, ISSN 1569-1993, 05/2019, Volume 18, Issue 3, pp. 420 - 424
The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the... 
Cystic fibrosis | Exercise tolerance | Longer-term treatment benefits | Daily physical activity | Lumacaftor–ivacaftor | RESPIRATORY SYSTEM | Lumacaftor-ivacaftor | Ivacaftor | Physical fitness | Adults | Exercise
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 08/2019, Volume 129, Issue 8, p. 3448
Cystic fibrosis (CF) is a multiorgan progressive genetic disease caused by loss of functional cystic fibrosis transmembrane conductance regulator (CFTR)... 
Cystic fibrosis | Ivacaftor | Genetic transcription | Protein binding
Journal Article
Anales de Pediatria, ISSN 1695-4033, 03/2019, Volume 90, Issue 3, pp. 148 - 156
Introduction: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and... 
Ivacaftor | Cystic fibrosis | Real-life
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 10/2018, Volume 379, Issue 17, pp. 1612 - 1620
This preclinical, phase 2 report shows that VX-445, a CFTR potentiator when administered with tezacaftor and ivacaftor, improved lung function and reduced... 
G551D MUTATION | MEDICINE, GENERAL & INTERNAL | TEZACAFTOR-IVACAFTOR | CFTR | Pyrazoles - therapeutic use | Forced Expiratory Volume - drug effects | Humans | Pyrrolidines - administration & dosage | Chloride Channel Agonists - therapeutic use | Male | Aminophenols - therapeutic use | Chloride Channel Agonists - adverse effects | Aminophenols - adverse effects | Pyrrolidines - pharmacology | Pyrrolidines - therapeutic use | Sweat - chemistry | Young Adult | Quinolones - adverse effects | Chlorides - metabolism | Quinolones - therapeutic use | Adult | Benzodioxoles - adverse effects | Female | Benzodioxoles - therapeutic use | Pyrazoles - pharmacology | Pyridines - therapeutic use | Pyridines - administration & dosage | Double-Blind Method | Genotype | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Chlorides - analysis | Pyrazoles - administration & dosage | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Alleles | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Indoles - therapeutic use | Pyridines - pharmacology | Mutation | Cystic Fibrosis - drug therapy | Drug Combinations | Cystic fibrosis | Ivacaftor | Dosage and administration | Drug therapy | Statistical analysis | Writers | Epithelial cells | Chloride transport | Recovery of function | Patients | Design | Proteins | Genotype & phenotype | Chloride | Collaboration | Conductance | Full text | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Pharmaceuticals
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 10/2018, Volume 379, Issue 17, pp. 1599 - 1611
This companion article to the VX-445 report shows that VX-659, a new CFTR potentiator, when administered with tezacaftor and ivacaftor improved lung function,... 
G551D MUTATION | MEDICINE, GENERAL & INTERNAL | IN-VITRO | CFTR POTENTIATOR | TEZACAFTOR-IVACAFTOR | Pyrazoles - therapeutic use | Forced Expiratory Volume - drug effects | Humans | Pyrrolidines - adverse effects | Chloride Channel Agonists - therapeutic use | Male | Aminophenols - therapeutic use | Chloride Channel Agonists - adverse effects | Aminophenols - adverse effects | Pyrrolidines - pharmacology | Pyrrolidines - therapeutic use | Sweat - chemistry | Young Adult | Quinolones - adverse effects | Quinolones - therapeutic use | Adult | Benzodioxoles - adverse effects | Female | Benzodioxoles - therapeutic use | Pyrazoles - adverse effects | Pyrazoles - pharmacology | Double-Blind Method | Cells, Cultured | Genotype | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Chlorides - analysis | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Alleles | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Indoles - therapeutic use | Mutation | Cystic Fibrosis - drug therapy | Drug Combinations | Cystic fibrosis | Ivacaftor | Genetic aspects | Dosage and administration | Drug therapy | Epithelial cells | Chloride transport | Clinical trials | Recovery of function | Patients | Proteins | Genotype & phenotype | Conductance | Full text | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Binding sites | Genotypes
Journal Article
International Journal of Research in Pharmaceutical Sciences, 2018, Volume 9, Issue 4, pp. 1169 - 1173
Journal Article
Scientific Reports, ISSN 2045-2322, 12/2019, Volume 9, Issue 1, pp. 7234 - 9
Several placebo-controlled trials have been recently published evaluating novel therapies targeting the defective CFTR protein. This systematic review examines... 
IN-VITRO | THERAPY | TEZACAFTOR-IVACAFTOR | MULTIDISCIPLINARY SCIENCES | PHE508DEL CFTR | MUTATION | DOUBLE-BLIND | F508DEL-CFTR | PHASE-3 | LUMACAFTOR/IVACAFTOR COMBINATION | POTENTIATOR | Clinical trials | Cystic fibrosis | Systematic review | Mutation | Cystic fibrosis transmembrane conductance regulator | Nonsense mutation
Journal Article
Paediatric Respiratory Reviews, ISSN 1526-0542, 04/2019, Volume 30, pp. 25 - 26
Journal Article
American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 06/2017, Volume 195, Issue 12, pp. 1617 - 1628
Journal Article
Pediatric Pulmonology, ISSN 8755-6863, 11/2019, Volume 54, Issue 11, pp. 1654 - 1654
Journal Article
Chest, ISSN 0012-3692, 02/2018, Volume 153, Issue 2, pp. 395 - 403
Ivacaftor produces significant clinical benefit in patients with cystic fibrosis (CF) with the G551D mutation. Prevalence of this mutation at the Cork CF... 
low-dose chest CT imaging | G551D | ivacaftor | cystic fibrosis
Journal Article
Pediatric Pulmonology, ISSN 8755-6863, 06/2019, Volume 54, Issue 6, pp. 672 - 674
Journal Article
Clinical Infectious Diseases, ISSN 1058-4838, 2015, Volume 60, Issue 5, pp. 703 - 712
Journal Article