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Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells

The airway epithelium contains ionocytes, a rare cell type with high expression of Forkhead Box I1 (FOXI1) transcription factor and Cystic Fibrosis Transmembrane conductance Regulator (CFTR), a chloride channel that is defective in cystic fibrosis (CF). Our aim was to verify if ionocyte development...

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Autores principales: Scudieri, Paolo, Musante, Ilaria, Venturini, Arianna, Guidone, Daniela, Genovese, Michele, Cresta, Federico, Caci, Emanuela, Palleschi, Alessandro, Poeta, Marco, Santamaria, Francesca, Ciciriello, Fabiana, Lucidi, Vincenzina, Galietta, Luis J. V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7565890/
https://www.ncbi.nlm.nih.gov/pubmed/32933106
http://dx.doi.org/10.3390/cells9092090
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author Scudieri, Paolo
Musante, Ilaria
Venturini, Arianna
Guidone, Daniela
Genovese, Michele
Cresta, Federico
Caci, Emanuela
Palleschi, Alessandro
Poeta, Marco
Santamaria, Francesca
Ciciriello, Fabiana
Lucidi, Vincenzina
Galietta, Luis J. V.
author_facet Scudieri, Paolo
Musante, Ilaria
Venturini, Arianna
Guidone, Daniela
Genovese, Michele
Cresta, Federico
Caci, Emanuela
Palleschi, Alessandro
Poeta, Marco
Santamaria, Francesca
Ciciriello, Fabiana
Lucidi, Vincenzina
Galietta, Luis J. V.
author_sort Scudieri, Paolo
collection PubMed
description The airway epithelium contains ionocytes, a rare cell type with high expression of Forkhead Box I1 (FOXI1) transcription factor and Cystic Fibrosis Transmembrane conductance Regulator (CFTR), a chloride channel that is defective in cystic fibrosis (CF). Our aim was to verify if ionocyte development is altered in CF and to investigate the relationship between ionocytes and CFTR-dependent chloride secretion. We collected nasal cells by brushing to determine ionocyte abundance. Nasal and bronchial cells were also expanded in vitro and reprogrammed to differentiated epithelia for morphological and functional studies. We found a relatively high (~3%) ionocyte abundance in ex vivo nasal samples, with no difference between CF and control individuals. In bronchi, ionocytes instead appeared very rarely as previously reported, thus suggesting a possible proximal–distal gradient in human airways. The difference between nasal and bronchial epithelial cells was maintained in culture, which suggests an epigenetic control of ionocyte development. In the differentiation phase of the culture procedure, we used two media that resulted in a different pattern of CFTR expression: confined to ionocytes or more broadly expressed. CFTR function was similar in both conditions, thus indicating that chloride secretion equally occurs irrespective of CFTR expression pattern.
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spelling pubmed-75658902020-10-26 Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells Scudieri, Paolo Musante, Ilaria Venturini, Arianna Guidone, Daniela Genovese, Michele Cresta, Federico Caci, Emanuela Palleschi, Alessandro Poeta, Marco Santamaria, Francesca Ciciriello, Fabiana Lucidi, Vincenzina Galietta, Luis J. V. Cells Article The airway epithelium contains ionocytes, a rare cell type with high expression of Forkhead Box I1 (FOXI1) transcription factor and Cystic Fibrosis Transmembrane conductance Regulator (CFTR), a chloride channel that is defective in cystic fibrosis (CF). Our aim was to verify if ionocyte development is altered in CF and to investigate the relationship between ionocytes and CFTR-dependent chloride secretion. We collected nasal cells by brushing to determine ionocyte abundance. Nasal and bronchial cells were also expanded in vitro and reprogrammed to differentiated epithelia for morphological and functional studies. We found a relatively high (~3%) ionocyte abundance in ex vivo nasal samples, with no difference between CF and control individuals. In bronchi, ionocytes instead appeared very rarely as previously reported, thus suggesting a possible proximal–distal gradient in human airways. The difference between nasal and bronchial epithelial cells was maintained in culture, which suggests an epigenetic control of ionocyte development. In the differentiation phase of the culture procedure, we used two media that resulted in a different pattern of CFTR expression: confined to ionocytes or more broadly expressed. CFTR function was similar in both conditions, thus indicating that chloride secretion equally occurs irrespective of CFTR expression pattern. MDPI 2020-09-13 /pmc/articles/PMC7565890/ /pubmed/32933106 http://dx.doi.org/10.3390/cells9092090 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Scudieri, Paolo
Musante, Ilaria
Venturini, Arianna
Guidone, Daniela
Genovese, Michele
Cresta, Federico
Caci, Emanuela
Palleschi, Alessandro
Poeta, Marco
Santamaria, Francesca
Ciciriello, Fabiana
Lucidi, Vincenzina
Galietta, Luis J. V.
Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title_full Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title_fullStr Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title_full_unstemmed Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title_short Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells
title_sort ionocytes and cftr chloride channel expression in normal and cystic fibrosis nasal and bronchial epithelial cells
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7565890/
https://www.ncbi.nlm.nih.gov/pubmed/32933106
http://dx.doi.org/10.3390/cells9092090
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