المؤلفون: Bouva, MJ, Dankert-Roelse, JE, van der Ploeg, CPB, Verschoof-Puite, RK, Zomer-van Ommen, DD, Gille, JJP, Jakobs, BS, Heijnen, MLA, de Winter-de Groot, KM

المصدر: In Journal of Cystic Fibrosis January 2024 23(1):120-125

المؤلفون: Zomer-van Ommen Dd, Peter van Mourik, Dompeling E, Gitte Berkers, Roukema J, de Winter-de Groot Km, Brunsveld Jb, Annelotte M. Vonk, Jans, Sylvia W.F. Suen, Sabine Michel, Weersink Ej, de Poel E, van der Ent Kk, Evelien Kruisselbrink, Marne C. Hagemeijer, Gerard H. Koppelman, Jeffrey M. Beekman, Vries R, Eijkemans Rj, van Panhuis H, H. Oppelaar, D. Muilwijk, van de Meer R, van der Eerden Mm

مصطلحات موضوعية: Forskolin, biology, business.industry, Disease, medicine.disease, Cystic fibrosis, Cystic fibrosis transmembrane conductance regulator, chemistry.chemical_compound, Liver disease, chemistry, Diabetes mellitus, Cancer research, biology.protein, Organoid, Biomarker (medicine), Medicine, business

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_________::60fbfd07b8d76c5c8b5befcc105028cc
https://doi.org/10.1101/2021.02.01.21250609

المؤلفون: Muilwijk D; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA, Utrecht, the Netherlands.; Department of Pulmonology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, the Netherlands., van Paridon TJ; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA, Utrecht, the Netherlands., van der Heijden DC; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA, Utrecht, the Netherlands., Faber-Bisschop BM; Dutch Cystic Fibrosis Foundation (NCFS), Dr. Albert Schweitzerweg 3a, 3744 MG, Baarn, the Netherlands., Zomer-van Ommen DD; Dutch Cystic Fibrosis Foundation (NCFS), Dr. Albert Schweitzerweg 3a, 3744 MG, Baarn, the Netherlands., Heijerman HGM; Department of Pulmonology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, the Netherlands., van der Ent CK; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA, Utrecht, the Netherlands.

المصدر: EClinicalMedicine [EClinicalMedicine] 2023 Jul 27; Vol. 62, pp. 102116. Date of Electronic Publication: 2023 Jul 27 (Print Publication: 2023).

نوع المنشور: Journal Article

بيانات الدورية: Publisher: The Lancet Country of Publication: England NLM ID: 101733727 Publication Model: eCollection Cited Medium: Internet ISSN: 2589-5370 (Electronic) Linking ISSN: 25895370 NLM ISO Abbreviation: EClinicalMedicine Subsets: PubMed not MEDLINE

المؤلفون: Muilwijk D; Department of Pediatric Pulmonology, University Medical Center Utrecht, loc. Wilhelmina Children's Hospital, Utrecht, The Netherlands., Zomer-van Ommen DD; Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands.; On behalf of the Dutch CF Registry Steering Group., Gulmans VAM; Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands.; On behalf of the Dutch CF Registry Steering Group., Eijkemans MJC; Department of Data Science and Biostatistics, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands., van der Ent CK; Department of Pediatric Pulmonology, University Medical Center Utrecht, loc. Wilhelmina Children's Hospital, Utrecht, The Netherlands.

مؤلفون مشاركون: Dutch Cystic Fibrosis Registry (NCFR) Steering Group:

المصدر: ERJ open research [ERJ Open Res] 2022 Nov 14; Vol. 8 (4). Date of Electronic Publication: 2022 Nov 14 (Print Publication: 2022).

نوع المنشور: Journal Article

بيانات الدورية: Publisher: European Respiratory Society Country of Publication: England NLM ID: 101671641 Publication Model: eCollection Cited Medium: Print ISSN: 2312-0541 (Print) Linking ISSN: 23120541 NLM ISO Abbreviation: ERJ Open Res Subsets: PubMed not MEDLINE

المؤلفون: Muilwijk D; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; These authors contributed equally to this work., de Poel E; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands.; These authors contributed equally to this work., van Mourik P; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., Suen SWF; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands., Vonk AM; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands., Brunsveld JE; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands., Kruisselbrink E; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands., Oppelaar H; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands., Hagemeijer MC; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Center for Lysosomal and Metabolic Diseases, Dept of Clinical Genetics, Erasmus University Medical Center, Rotterdam, The Netherlands., Berkers G; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., de Winter-de Groot KM; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., Heida-Michel S; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., Jans SR; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., van Panhuis H; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands., van der Eerden MM; Dept of Pulmonology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands., van der Meer R; Haga Teaching Hospital, The Hague, The Netherlands., Roukema J; Radboud University Medical Center, Radboud Institute for Health Sciences, Nijmegen, The Netherlands., Dompeling E; Maastricht University Medical Center, Maastricht, The Netherlands., Weersink EJM; Amsterdam University Medical Center, location AMC, Amsterdam, The Netherlands., Koppelman GH; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Pulmonology and Pediatric Allergology, Groningen, The Netherlands.; University of Groningen, University Medical Center Groningen, Groningen Research Institute for Asthma and COPD (GRIAC), Groningen, The Netherlands., Vries R; Hubrecht Organoid Technology (HUB), Utrecht, The Netherlands., Zomer-van Ommen DD; Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands., Eijkemans MJC; Dept of Data Science and Biostatistics, Julius Center for Health Sciences and Primary Care, University Medical Center, Utrecht University, Utrecht, The Netherlands., van der Ent CK; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands.; C.K. van der Ent and J.M. Beekman contributed equally to this article as lead authors and supervised the work., Beekman JM; Dept of Pediatric Respiratory Medicine, Wilhelmina Children's Hospital, University Medical Center, Utrecht University, Utrecht, The Netherlands J.Beekman@umcutrecht.nl.; Regenerative Medicine Utrecht, University Medical Center, Utrecht University, Utrecht, The Netherlands.; Center for Living Technologies, University Medical Center, Utrecht, The Netherlands.; C.K. van der Ent and J.M. Beekman contributed equally to this article as lead authors and supervised the work.

المصدر: The European respiratory journal [Eur Respir J] 2022 Aug 18; Vol. 60 (2). Date of Electronic Publication: 2022 Aug 18 (Print Publication: 2022).

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

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

مواضيع طبية MeSH: Cystic Fibrosis*/complications , Cystic Fibrosis*/genetics , Exocrine Pancreatic Insufficiency*/complications, Biomarkers ; Colforsin/pharmacology ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Disease Progression ; Humans ; Mutation ; Organoids

المؤلفون: Sachs N; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Papaspyropoulos A; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Zomer-van Ommen DD; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Heo I; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Böttinger L; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Klay D; St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands., Weeber F; The Netherlands Cancer Institute, Amsterdam, The Netherlands., Huelsz-Prince G; FOM Institute AMOLF, Amsterdam, The Netherlands., Iakobachvili N; Maastricht University, Maastricht, The Netherlands., Amatngalim GD; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., de Ligt J; UMC Utrecht, Utrecht, The Netherlands., van Hoeck A; UMC Utrecht, Utrecht, The Netherlands., Proost N; Mouse Clinic for Cancer and Aging (MCCA) Preclinical Intervention Unit, The Netherlands Cancer Institute, Amsterdam, The Netherlands., Viveen MC; UMC Utrecht, Utrecht, The Netherlands., Lyubimova A; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Teeven L; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Derakhshan S; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Korving J; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Begthel H; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Dekkers JF; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Kumawat K; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Ramos E; Hubrecht Organoid Technology, Utrecht, The Netherlands., van Oosterhout MF; St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands., Offerhaus GJ; UMC Utrecht, Utrecht, The Netherlands., Wiener DJ; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Olimpio EP; FOM Institute AMOLF, Amsterdam, The Netherlands., Dijkstra KK; The Netherlands Cancer Institute, Amsterdam, The Netherlands., Smit EF; The Netherlands Cancer Institute, Amsterdam, The Netherlands., van der Linden M; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Jaksani S; Hubrecht Organoid Technology, Utrecht, The Netherlands., van de Ven M; Mouse Clinic for Cancer and Aging (MCCA) Preclinical Intervention Unit, The Netherlands Cancer Institute, Amsterdam, The Netherlands., Jonkers J; Mouse Clinic for Cancer and Aging (MCCA) Preclinical Intervention Unit, The Netherlands Cancer Institute, Amsterdam, The Netherlands., Rios AC; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., Voest EE; The Netherlands Cancer Institute, Amsterdam, The Netherlands., van Moorsel CH; St. Antonius Hospital Nieuwegein, Nieuwegein, The Netherlands., van der Ent CK; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Cuppen E; UMC Utrecht, Utrecht, The Netherlands., van Oudenaarden A; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands., Coenjaerts FE; UMC Utrecht, Utrecht, The Netherlands., Meyaard L; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Bont LJ; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Peters PJ; Maastricht University, Maastricht, The Netherlands., Tans SJ; FOM Institute AMOLF, Amsterdam, The Netherlands., van Zon JS; FOM Institute AMOLF, Amsterdam, The Netherlands., Boj SF; Hubrecht Organoid Technology, Utrecht, The Netherlands., Vries RG; Hubrecht Organoid Technology, Utrecht, The Netherlands., Beekman JM; Wilhelmina Children's Hospital and UMC Utrecht, Utrecht, The Netherlands., Clevers H; Oncode Institute, Hubrecht Institute-KNAW and UMC Utrecht, Utrecht, The Netherlands h.clevers@hubrecht.eu.; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.

المصدر: The EMBO journal [EMBO J] 2019 Feb 15; Vol. 38 (4). Date of Electronic Publication: 2019 Jan 14.

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

بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 8208664 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1460-2075 (Electronic) Linking ISSN: 02614189 NLM ISO Abbreviation: EMBO J Subsets: MEDLINE

مواضيع طبية MeSH: Carcinoma, Non-Small-Cell Lung/*pathology , Cystic Fibrosis/*pathology , Epithelial Cells/*pathology , Organ Culture Techniques/*methods , Organoids/*pathology , Respiratory Syncytial Virus Infections/*pathology , Respiratory System/*pathology, Animals ; Carcinoma, Non-Small-Cell Lung/drug therapy ; Carcinoma, Non-Small-Cell Lung/metabolism ; Cells, Cultured ; Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Disease Models, Animal ; Drug Screening Assays, Antitumor ; Epithelial Cells/metabolism ; Female ; Humans ; Lung Neoplasms/drug therapy ; Lung Neoplasms/metabolism ; Lung Neoplasms/pathology ; Male ; Mice ; Mice, Inbred NOD ; Mice, SCID ; Organoids/metabolism ; Respiratory Syncytial Virus Infections/virology ; Respiratory Syncytial Viruses/isolation & purification ; Respiratory System/metabolism ; Xenograft Model Antitumor Assays

المؤلفون: Zomer-van Ommen DD; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., de Poel E; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., Kruisselbrink E; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., Oppelaar H; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., Vonk AM; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., Janssens HM; Department of Pediatric Pulmonology, Erasmus Medical Centre, Sophia Children's Hospital, Rotterdam, The Netherlands., van der Ent CK; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands., Hagemeijer MC; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands., Beekman JM; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands; Regenerative Medicine Center Utrecht, University Medical Centre Utrecht, Utrecht, The Netherlands. Electronic address: jbeekman@umcutrecht.nl.

المصدر: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2018 May; Vol. 17 (3), pp. 316-324. Date of Electronic Publication: 2018 Mar 13.

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*/genetics , Cystic Fibrosis*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism, Epithelial Cells/*metabolism , Ion Transport/*physiology, Cells, Cultured ; Humans ; Models, Biological ; Mutation ; Organoids/physiology ; Rectum

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; Department of Pediatric Pulmonology, University Medical Centre Utrecht , Lundlaan 6, 3508 GA Utrecht, The Netherlands., Pukin AV; Department of Chemical Biology & Drug Discovery, Utrecht Institute for Pharmaceutical Sciences, Utrecht University , P.O. Box 80082, 3508 TB Utrecht, The Netherlands., Fu O; Department of Chemical Biology & Drug Discovery, Utrecht Institute for Pharmaceutical Sciences, Utrecht University , P.O. Box 80082, 3508 TB Utrecht, The Netherlands., Quarles van Ufford LH; Department of Chemical Biology & Drug Discovery, Utrecht Institute for Pharmaceutical Sciences, Utrecht University , P.O. Box 80082, 3508 TB Utrecht, The Netherlands., Janssens HM; Department of Pediatric Pulmonology, Erasmus Medical Center/Sophia Children's Hospital , Wytemaweg 80, 3015 CN Rotterdam, The Netherlands., Beekman JM; Department of Pediatric Pulmonology, University Medical Centre Utrecht , Lundlaan 6, 3508 GA Utrecht, The Netherlands., Pieters RJ; Department of Chemical Biology & Drug Discovery, Utrecht Institute for Pharmaceutical Sciences, Utrecht University , P.O. Box 80082, 3508 TB Utrecht, The Netherlands.

المصدر: Journal of medicinal chemistry [J Med Chem] 2016 Jul 28; Vol. 59 (14), pp. 6968-72. Date of Electronic Publication: 2016 Jul 07.

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

بيانات الدورية: Publisher: American Chemical Society Country of Publication: United States NLM ID: 9716531 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1520-4804 (Electronic) Linking ISSN: 00222623 NLM ISO Abbreviation: J Med Chem Subsets: MEDLINE

مواضيع طبية MeSH: Antitoxins/*pharmacology , Cholera Toxin/*antagonists & inhibitors , Intestines/*drug effects , Organoids/*drug effects, Antitoxins/chemistry ; Dose-Response Relationship, Drug ; Humans ; Molecular Structure ; Structure-Activity Relationship

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