Display options
Cite
Collin AM, Lecocq M, Detry B, et al. Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis. J Cyst Fibros. 2021;20(6):e129-e139doi: 10.1016/j.jcf.2021.09.019.
Collin, A. M., Lecocq, M., Detry, B., Carlier, F. M., Bouzin, C., de Sany, P., Hoton, D., Verleden, S., Froidure, A., Pilette, C., & Gohy, S. (2021). Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 20(6), e129-e139. https://doi.org/10.1016/j.jcf.2021.09.019
Collin, Amandine M, et al. "Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis." Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society vol. 20,6 (2021): e129-e139. doi: https://doi.org/10.1016/j.jcf.2021.09.019
Collin AM, Lecocq M, Detry B, Carlier FM, Bouzin C, de Sany P, Hoton D, Verleden S, Froidure A, Pilette C, Gohy S. Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis. J Cyst Fibros. 2021 Nov;20(6):e129-e139. doi: 10.1016/j.jcf.2021.09.019. Epub 2021 Oct 15. PMID: 34657818.
Copy Download .nbib Format: NLM
Share it on

J Cyst Fibros. 2021 Nov;20(6):e129-e139. doi: 10.1016/j.jcf.2021.09.019. Epub 2021 Oct 15.

Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

Amandine M Collin, Marylène Lecocq, Bruno Detry, François M Carlier, Caroline Bouzin, Philippe de Sany, Delphine Hoton, Stijn Verleden, Antoine Froidure, Charles Pilette, Sophie Gohy

Affiliations

  1. Pole of Pneumology, ENT and Dermatology, Institute of Experimental & Clinical Research, Université catholique de Louvain (UCLouvain), Brussels, Belgium.
  2. IREC Imaging Platform, Institute of Experimental & Clinical Research, Université catholique de Louvain (UCLouvain), Brussels, Belgium.
  3. Pole of Microbiology, Institute of Experimental & Clinical Research, Université catholique de Louvain (UCLouvain), Brussels, Belgium.
  4. Department of Pathology, Cliniques universitaires Saint-Luc, Brussels, Belgium.
  5. Lung Transplant Unit, Division of Respiratory Disease, Department of chronic disease, metabolism and aging, Katholieke Universiteit Leuven, Leuven, Belgium.
  6. Pole of Pneumology, ENT and Dermatology, Institute of Experimental & Clinical Research, Université catholique de Louvain (UCLouvain), Brussels, Belgium; Department of Pneumology, Cliniques universitaires Saint-Luc, Brussels, Belgium.
  7. Pole of Pneumology, ENT and Dermatology, Institute of Experimental & Clinical Research, Université catholique de Louvain (UCLouvain), Brussels, Belgium; Department of Pneumology, Cliniques universitaires Saint-Luc, Brussels, Belgium; Centre de référence pour la mucoviscidose, Cliniques universitaires Saint-Luc, Brussels, Belgium. Electronic address: [email protected].

PMID: 34657818 DOI: 10.1016/j.jcf.2021.09.019

Abstract

BACKGROUND: In cystic fibrosis, the respiratory epithelium is the target tissue of both the genetic abnormality of the disease and of external aggressions, notably by pathogens (Pseudomonas aeruginosa). A detailed characterisation of the cystic fibrosis bronchial epithelium is however lacking, as most previous studies focused on the nasal epithelium or on cell lines. This study aimed to characterise the abnormal phenotype and epithelial-to-mesenchymal transition in cystic fibrosis bronchial epithelium and to evaluate in cell cultures whether abnormalities persist ex vivo.

METHODS: Explant lung tissues (n = 44) were assessed for bronchial epithelial cell phenotyping by immunostaining. Human bronchial epithelial cells were derived from basal cells isolated from cystic fibrosis patients or control donors and cultured in air-liquid interface for 2, 4 or 6 weeks.

RESULTS: Enhanced mucin 5AC and decreased β-tubulin expression were observed in cystic fibrosis airways reflecting a decreased ciliated/goblet cell ratio, associated with increased number of vimentin-positive cells, indicating epithelial-to-mesenchymal transition process. These features were recapitulated in vitro, in cystic fibrosis-derived reconstituted epithelium. However, they were not induced by CFTR inhibition or Pseudomonas infection, and most abnormalities tended to disappear in long-term culture (6 weeks) except for increased fibronectin release, an epithelial-to-mesenchymal transition marker.

CONCLUSIONS: This study provides new insights into airway epithelial changes in cystic fibrosis, which are imprinted through an acquired mechanism that we could not relate to CFTR function.

Copyright © 2021. Published by Elsevier B.V.

Keywords: Epithelial differentiation; Epithelial-to-mesenchymal transition; Respiratory epithelium

Conflict of interest statement

Declaration of Competing Interest Authors declare no conflict of interest.

Publication Types