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PLoS pathogens, ISSN 1553-7374, 2018, Volume 14, Issue 12, p. e1007453
...Author(s): Julio Diaz Caballero 1, Shawn T. Clark 2,3, Pauline W. Wang 4, Sylva L. Donaldson 4, Bryan Coburn 5, D. Elizabeth Tullis 6, Yvonne C. W. Yau 3,7... 
INFECTIONS | ANTIBIOTIC-RESISTANCE | MICROBIOLOGY | EFFLUX-PUMP | BETA-LACTAM | VIROLOGY | PSEUDOMONAS-AERUGINOSA | ADAPTATION | CYSTIC-FIBROSIS | MUTATIONS | EPIDEMIOLOGY | PARASITOLOGY | MOLECULAR-MECHANISMS | Genome-Wide Association Study | Genes, Bacterial - genetics | Genetic Variation - drug effects | Recombination, Genetic | Humans | Burkholderia Infections - genetics | Burkholderia cepacia complex - genetics | Drug Resistance, Bacterial - genetics | Evolution, Molecular | Drug resistance in microorganisms | Gram-negative bacterial infections | Genome-wide association studies | Usage | Research | Risk factors | Recombination | Thoracic surgery | Transcription | Laboratories | Syngeneic grafts | Genomics | Virulence | Lung transplantation | Chronic infection | Association analysis | Amides | Aminoglycosides | Population studies | Genomes | Infections | Drug resistance | Antiinfectives and antibacterials | Quinolones | Health care networks | Antibiotic resistance | Phylogenetics | Evolution | Population | Sampling | Bioinformatics | Supervision | β-Lactam antibiotics | Cystic fibrosis | Antimicrobial agents | Loci | Morbidity | Medicine | Genetic variance | Population (statistical) | Hospitals | Infectious diseases | Antibiotics | Fibrosis | Sputum | Software | Antimicrobial resistance | Mutation | Polymorphism
Journal Article
The New England journal of medicine, ISSN 1533-4406, 2017, Volume 377, Issue 21, pp. 2024 - 2035
Journal Article
NPJ biofilms and microbiomes, ISSN 2055-5008, 2019, Volume 5, Issue 1, pp. 4 - 12
... Layeghifard1, Hannah Li2, Pauline W. Wang1,3, Sylva L. Donaldson3, Bryan Coburn4, Shawn T. Clark5, Julio Diaz Caballero1, Yu Zhang5, D. Elizabeth Tullis6, Yvonne C. W... 
INFECTIONS | BIOTECHNOLOGY & APPLIED MICROBIOLOGY | CHAIN FATTY-ACIDS | AIRWAY MICROBIOTA | STABILITY | INFLAMMATION | RESILIENCE | FERMENTATION | ANAEROBIC-BACTERIA | MICROBIOLOGY | DIVERSITY | GUT MICROBIOTA | Community structure | Respiratory tract | Anaerobic bacteria | Microbiota | Respiratory function | Lung diseases | Sputum | Microbiomes | Cystic fibrosis | Abundance
Journal Article
The New England journal of medicine, ISSN 1533-4406, 2011, Volume 365, Issue 18, pp. 1663 - 1672
Journal Article
The New England journal of medicine, ISSN 1533-4406, 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 | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Pharmaceuticals
Journal Article
The New England journal of medicine, ISSN 1533-4406, 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 | Cystic fibrosis transmembrane conductance regulator | Protein transport | Sweat | Binding sites | Genotypes
Journal Article
American journal of respiratory and critical care medicine, ISSN 1535-4970, 2018, Volume 197, Issue 2, pp. 214 - 224
Rationale: Tezacaftor (formerly VX-661) is an investigational small molecule that improves processing and trafficking of the cystic fibrosis transmembrane... 
Sweat chloride | Cystic fibrosis transmembrane conductance regulator corrector | Forced expiratory volume | CFTR modulator | EFFICACY | SAFETY | LUMACAFTOR-IVACAFTOR | IDENTIFICATION | INVESTIGATIONAL CFTR CORRECTOR | TRANSMEMBRANE CONDUCTANCE REGULATOR | G551D MUTATION | F508DEL-CFTR MUTATION | GENE | RESPIRATORY SYSTEM | cystic fibrosis transmembrane conductance regulator corrector | PHE508DEL CFTR | forced expiratory volume | sweat chloride | CRITICAL CARE MEDICINE | Prognosis | Humans | Male | Benzodioxoles - administration & dosage | Aminophenols - adverse effects | Indoles - administration & dosage | Dose-Response Relationship, Drug | Young Adult | Quinolones - adverse effects | Adult | Benzodioxoles - adverse effects | Female | Drug Therapy, Combination | Molecular Targeted Therapy - methods | Severity of Illness Index | Double-Blind Method | Drug Administration Schedule | Risk Assessment | Administration, Oral | Treatment Outcome | Maximum Tolerated Dose | Quinolones - administration & dosage | Indoles - adverse effects | Cystic Fibrosis - genetics | Adolescent | Cystic Fibrosis - diagnosis | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Respiratory Function Tests | Aminophenols - administration & dosage | Cystic Fibrosis - drug therapy | Proteins | Pediatrics | Chloride | Cysts | Cystic fibrosis | Mutation | Kinases | Patients | Drug dosages | Original
Journal Article