دورية أكاديمية
أعرض في EDS

Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis.

التفاصيل البيبلوغرافية
العنوان: Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis.
المؤلفون: Rodenburg LW; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands. l.w.rodenburg-2@umcutrecht.nl.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands. l.w.rodenburg-2@umcutrecht.nl., Metzemaekers M; Department of Pulmonary Medicine, Erasmus University Medical Center, 3015 CE, Rotterdam, The Netherlands.; Department of Cell Biology, Erasmus University Medical Center, 3015 CE, Rotterdam, The Netherlands., van der Windt IS; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands., Smits SMA; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands., den Hertog-Oosterhoff LA; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands., Kruisselbrink E; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands., Brunsveld JE; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands., Michel S; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands., de Winter-de Groot KM; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands., van der Ent CK; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands., Stadhouders R; Department of Pulmonary Medicine, Erasmus University Medical Center, 3015 CE, Rotterdam, The Netherlands.; Department of Cell Biology, Erasmus University Medical Center, 3015 CE, Rotterdam, The Netherlands., Beekman JM; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands.; Centre for Living Technologies, Alliance TU/e, WUR, UU, UMC Utrecht, 3584 CB, Utrecht, The Netherlands., Amatngalim GD; Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Member of ERN-LUNG, 3584 EA, Utrecht, The Netherlands.; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, 3584 CT, Utrecht, The Netherlands.
المصدر: Scientific reports [Sci Rep] 2023 Oct 30; Vol. 13 (1), pp. 18573. Date of Electronic Publication: 2023 Oct 30.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : Nature Publishing Group, copyright 2011-
مواضيع طبية MeSH: Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism, Humans ; Cells, Cultured ; Respiratory Mucosa/metabolism ; Nose ; Epithelial Cells/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism
مستخلص: The nasal and bronchial epithelium are unified parts of the respiratory tract that are affected in the monogenic disorder cystic fibrosis (CF). Recent studies have uncovered that nasal and bronchial tissues exhibit intrinsic variability, including differences in mucociliary cell composition and expression of unique transcriptional regulatory proteins which relate to germ layer origin. In the present study, we explored whether intrinsic differences between nasal and bronchial epithelial cells persist in cell cultures and affect epithelial cell functioning in CF. Comparison of air-liquid interface (ALI) differentiated epithelial cells from subjects with CF revealed distinct mucociliary differentiation states of nasal and bronchial cultures. Moreover, using RNA sequencing we identified cell type-specific signature transcription factors in differentiated nasal and bronchial epithelial cells, some of which were already poised for expression in basal progenitor cells as evidenced by ATAC sequencing. Analysis of differentiated nasal and bronchial epithelial 3D organoids revealed distinct capacities for fluid secretion, which was linked to differences in ciliated cell differentiation. In conclusion, we show that unique phenotypical and functional features of nasal and bronchial epithelial cells persist in cell culture models, which can be further used to investigate the effects of tissue-specific features on upper and lower respiratory disease development in CF.
(© 2023. The Author(s).)
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المشرفين على المادة: 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
تواريخ الأحداث: Date Created: 20231031 Date Completed: 20231101 Latest Revision: 20231107
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC10616285
DOI: 10.1038/s41598-023-45201-4
PMID: 37903789
قاعدة البيانات: MEDLINE
الوصف
تدمد:2045-2322
DOI:10.1038/s41598-023-45201-4