A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
2011· article· en· New England Journal of Medicine· Medicine
aff
2 174
citations
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Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial
2019· article· en· The Lancet· Medicine
affsans résumé
1 367
citations
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene
2013· article· en· Nature Genetics· Medicine
affsans résumé
625
citations
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations
2016· article· en· Molecular Biology of the Cell· Medicine
afffund
590
citations
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
2011· article· en· Thorax· Medicine
afffund
496
citations
Neutrophil Elastase Activity Is Associated with Exacerbations and Lung Function Decline in Bronchiectasis
2017· article· en· American Journal of Respiratory and Critical Care Medicine· Medicine
fundno aff
414
citations
CFTR: folding, misfolding and correcting the ΔF508 conformational defect
2011· review· en· Trends in Molecular Medicine· Medicine
afffundsans résumé
382
citations
Effect of Azithromycin on Pulmonary Function in Patients With Cystic Fibrosis Uninfected With <emph type="ital">Pseudomonas aeruginosa</emph><subtitle>A Randomized Controlled Trial</subtitle>
2010· article· en· JAMA· Medicine
aboutno aff
331
citations
Directed differentiation of cholangiocytes from human pluripotent stem cells
2015· article· en· Nature Biotechnology· Medicine
afffundsans résumé
314
citations
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent Manner
2012· article· en· Journal of Biological Chemistry· Medicine
afffund
308
citations
Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination
2020· article· en· JCI Insight· Medicine
afffund
261
citations
The Burkholderia Genome Database: facilitating flexible queries and comparative analyses
2008· article· en· Bioinformatics· Medicine
afffund
252
citations
Alcohol Disrupts Levels and Function of the Cystic Fibrosis Transmembrane Conductance Regulator to Promote Development of Pancreatitis
2014· article· en· Gastroenterology· Medicine
aff
199
citations
Inhaled Hypertonic Saline in Infants and Children Younger Than 6 Years With Cystic Fibrosis
2012· article· en· JAMA· Medicine
affabout
191
citations
Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis
2015· article· en· American Journal of Respiratory and Critical Care Medicine· Medicine
aff
179
citations
User Preferences and Design Recommendations for an mHealth App to Promote Cystic Fibrosis Self-Management
2014· article· en· JMIR mhealth and uhealth· Medicine
venueno aff
169
citations
Clinical utilization of genomics data produced by the international Pseudomonas aeruginosa consortium
2015· article· en· Frontiers in Microbiology· Biochemistry, Genetics and Molecular Biology
afffund
160
citations
Cooperative Assembly and Misfolding of CFTR Domains In Vivo
2009· article· en· Molecular Biology of the Cell· Medicine
afffund
149
citations
Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study
2022· article· en· American Journal of Respiratory and Critical Care Medicine· Medicine
aff
140
citations
Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosis
2015· article· en· European Respiratory Journal· Medicine
aff
116
citations
Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial
2019· article· en· The Lancet Respiratory Medicine· Medicine
afffundsans résumé
115
citations
NMR evidence for differential phosphorylation‐dependent interactions in WT and ΔF508 CFTR
2009· article· en· The EMBO Journal· Medicine
afffundsans résumé
109
citations
Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR
2021· article· en· Journal of Cystic Fibrosis· Medicine
afffundsans résumé
103
citations
Normative data for multiple breath washout outcomes in school-aged Caucasian children
2019· article· en· European Respiratory Journal· Medicine
aff
98
citations
Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR
2011· article· en· Chemistry & Biology· Medicine
afffundsans résumé
98
citations