المؤلفون: Vijftigschild LA; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Berkers G; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Dekkers JF; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Zomer-van Ommen DD; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Matthes E; CF Translational Research Centre, Dept of Physiology, McGill University, Montréal, QC, Canada., Kruisselbrink E; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands., Vonk A; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands., Hensen CE; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Heida-Michel S; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Geerdink M; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Janssens HM; Dept of Pediatric Pulmonology, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands., van de Graaf EA; Dept of Pulmonology, University Medical Center, Utrecht, The Netherlands., Bronsveld I; Dept of Pulmonology, University Medical Center, Utrecht, The Netherlands., de Winter-de Groot KM; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Majoor CJ; Dept of Respiratory Medicine, Academic Medical Center, Amsterdam, The Netherlands., Heijerman HG; Dept of Pulmonology and Cystic Fibrosis, Haga Teaching Hospital, The Hague, The Netherlands., de Jonge HR; Dept of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands., Hanrahan JW; CF Translational Research Centre, Dept of Physiology, McGill University, Montréal, QC, Canada., van der Ent CK; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Beekman JM; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Regenerative Medicine Center Utrecht, University Medical Center, Utrecht, The Netherlands jbeekman@umcutrecht.nl.

المصدر: The European respiratory journal [Eur Respir J] 2016 Sep; Vol. 48 (3), pp. 768-79. Date of Electronic Publication: 2016 Jul 28.

نوع المنشور: Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: European Respiratory Society Country of Publication: England NLM ID: 8803460 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1399-3003 (Electronic) Linking ISSN: 09031936 NLM ISO Abbreviation: Eur Respir J Subsets: MEDLINE

مواضيع طبية MeSH: Adrenergic beta-2 Receptor Agonists/*pharmacology , Cystic Fibrosis/*drug therapy , Cystic Fibrosis/*metabolism , Cystic Fibrosis Transmembrane Conductance Regulator/*metabolism, Administration, Oral ; Albuterol/administration & dosage ; Biological Assay ; Bronchi/pathology ; Cell Line ; Cells, Cultured ; Chlorides/chemistry ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Drug Evaluation, Preclinical ; Epithelial Cells/metabolism ; Epithelium/metabolism ; Humans ; Mutation ; Nasal Mucosa/drug effects ; Nasal Mucosa/metabolism ; Organoids ; Pilot Projects ; Respiratory System/metabolism ; Signal Transduction

المؤلفون: Zomer-van Ommen DD; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands., Vijftigschild LA; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands., Kruisselbrink E; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands., Vonk AM; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands., Dekkers JF; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands., Janssens HM; Dept of Pediatrics, Sophia Children's Hospital/Erasmus MC-University Medical Center, Rotterdam, The Netherlands., de Winter-de Groot KM; Dept of Pediatric Pulmonology, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands., van der Ent CK; Dept of Pediatric Pulmonology, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands., Beekman JM; Laboratory of Translational Immunology, UMC Utrecht, The Netherlands; Dept of Pediatric Pulmonology, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands. Electronic address: J.Beekman@umcutrecht.nl.

المصدر: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2016 Mar; Vol. 15 (2), pp. 158-62. Date of Electronic Publication: 2015 Aug 05.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

مواضيع طبية MeSH: Codon, Nonsense/*drug effects , Cystic Fibrosis/*drug therapy , Cystic Fibrosis Transmembrane Conductance Regulator/*drug effects , Gentamicins/*therapeutic use , Organoids/*cytology , Oxadiazoles/*therapeutic use, Cells, Cultured ; Coccidiostats/therapeutic use ; Cystic Fibrosis/genetics ; Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; RNA/genetics

المؤلفون: Dekkers R; Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., Vijftigschild LA; Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., Vonk AM; Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., Kruisselbrink E; Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., de Winter-de Groot KM; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., Janssens HM; Department of Pediatric Pulmonology, Erasmus University Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands., van der Ent CK; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands., Beekman JM; Laboratory of Translational Immunology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands. Electronic address: JBeekman@umcutrecht.nl.

المصدر: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2015 Mar; Vol. 14 (2), pp. 178-81. Date of Electronic Publication: 2014 Nov 06.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/physiopathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Intestinal Mucosa*/metabolism , Intestines*/pathology , Organoids*/drug effects , Organoids*/metabolism, Aminophenols/*pharmacokinetics , Quinolones/*pharmacokinetics, Antimutagenic Agents/pharmacokinetics ; Biological Assay ; Drug Monitoring/methods ; Humans ; Mutation/drug effects ; Treatment Outcome

المؤلفون: van de Weert-van Leeuwen PB; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands; Centre for Molecular and Cellular Intervention, University Medical Centre Utrecht, Utrecht, The Netherlands. Electronic address: p.b.vanleeuwen-5@umcutrecht.nl., Hulzebos HJ; Child Development & Exercise Centre, Cystic Fibrosis Centre, University Medical Centre Utrecht, Utrecht, The Netherlands., Werkman MS; Child Development & Exercise Centre, Cystic Fibrosis Centre, University Medical Centre Utrecht, Utrecht, The Netherlands., Michel S; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands., Vijftigschild LA; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands; Centre for Molecular and Cellular Intervention, University Medical Centre Utrecht, Utrecht, The Netherlands., van Meegen MA; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands; Centre for Molecular and Cellular Intervention, University Medical Centre Utrecht, Utrecht, The Netherlands., van der Ent CK; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands., Beekman JM; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands; Centre for Molecular and Cellular Intervention, University Medical Centre Utrecht, Utrecht, The Netherlands., Arets HG; Department of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands.

المصدر: Respiratory medicine [Respir Med] 2014 Mar; Vol. 108 (3), pp. 445-52. Date of Electronic Publication: 2013 Aug 28.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis/*complications , Cystic Fibrosis/*physiopathology , Exercise/*physiology , Exercise Tolerance/*physiology , Pseudomonas Infections/*complications, Adolescent ; Child ; Chronic Disease ; Cystic Fibrosis/immunology ; Humans ; Immunoglobulin G/blood ; Oxygen Consumption/physiology ; Pseudomonas aeruginosa/isolation & purification

المؤلفون: van Meegen MA; Department of Pediatric Pulmonology, University Medical Centre Utrecht, The Netherlands., Terheggen SW, Koymans KJ, Vijftigschild LA, Dekkers JF, van der Ent CK, Beekman JM

المصدر: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2013 Sep; Vol. 12 (5), pp. 487-96. Date of Electronic Publication: 2013 Jan 11.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

مواضيع طبية MeSH: Mutation*, Antibodies, Monoclonal/*immunology , Cystic Fibrosis Transmembrane Conductance Regulator/*genetics , Cystic Fibrosis Transmembrane Conductance Regulator/*immunology, Cells, Cultured ; Humans

المؤلفون: van Wandelen LT; Medicinal Chemistry and Chemical Biology, Faculty of Science, Utrecht University , Universiteitsweg 99, 3584 CG, Utrecht, The Netherlands., van Ameijde J, Ismail-Ali AF, van Ufford HC, Vijftigschild LA, Beekman JM, Martin NI, Ruijtenbeek R, Liskamp RM

المصدر: ACS chemical biology [ACS Chem Biol] 2013 Jul 19; Vol. 8 (7), pp. 1479-87. Date of Electronic Publication: 2013 May 08.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: American Chemical Society Country of Publication: United States NLM ID: 101282906 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1554-8937 (Electronic) Linking ISSN: 15548929 NLM ISO Abbreviation: ACS Chem Biol Subsets: MEDLINE

مواضيع طبية MeSH: Cell-Penetrating Peptides*/chemical synthesis , Cell-Penetrating Peptides*/chemistry , Cell-Penetrating Peptides*/pharmacokinetics , Drug Delivery Systems*, Protein Kinase Inhibitors/*pharmacokinetics, Amino Acid Sequence ; Binding Sites ; Binding, Competitive ; HeLa Cells ; Humans ; Inhibitory Concentration 50 ; Microscopy, Confocal ; Models, Biological ; Models, Molecular ; Protein Kinase Inhibitors/chemical synthesis ; Protein Kinase Inhibitors/chemistry ; Substrate Specificity

المؤلفون: Vijftigschild LA; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands., van der Ent CK, Beekman JM

المصدر: Cytometry. Part A : the journal of the International Society for Analytical Cytology [Cytometry A] 2013 Jun; Vol. 83 (6), pp. 576-84. Date of Electronic Publication: 2013 Mar 05.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: Wiley-Liss Country of Publication: United States NLM ID: 101235694 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1552-4930 (Electronic) Linking ISSN: 15524922 NLM ISO Abbreviation: Cytometry A Subsets: MEDLINE

مواضيع طبية MeSH: Cystic Fibrosis Transmembrane Conductance Regulator/*metabolism, Animals ; Bacterial Proteins ; Biological Transport/drug effects ; Cell Line ; Colforsin/pharmacology ; Cricetinae ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Flow Cytometry ; Gene Expression ; Genes, Reporter ; Genistein/pharmacology ; Humans ; Luminescent Proteins ; Mutation ; Spectrometry, Fluorescence