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Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?
- Source :
- Annual Review of Pathology: Mechanisms of Disease. 17:23-46
- Publication Year :
- 2022
- Publisher :
- Annual Reviews, 2022.
-
Abstract
- Cystic fibrosis (CF) is caused by defects in an anion channel, the cystic fibrosis transmembrane conductance regulator (CFTR). Recently, a new airway epithelial cell type has been discovered and dubbed the pulmonary ionocyte. Unexpectedly, these ionocytes express higher levels of CFTR than any other airway epithelial cell type. However, ionocytes are not the sole CFTR-expressing airway epithelial cells, and CF-associated disease genes are in fact expressed in multiple airway epithelial cell types. The experimental depletion of ionocytes perturbs epithelial physiology in the mouse trachea, but the role of these rare cells in the pathogenesis of human CF remains mysterious. Ionocytes have been described in diverse tissues (kidney and inner ear) and species (frog and fish). We draw on these prior studies to suggest potential roles of airway ionocytes in health and disease. A complete understanding of ionocytes in the mammalian airway will ultimately depend on cell type-specific genetic manipulation Expected final online publication date for the Annual Review of Pathology: Mechanisms of Disease, Volume 17 is January 2022. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.
- Subjects :
- Cystic Fibrosis
Cell
Cystic Fibrosis Transmembrane Conductance Regulator
Epithelial Cells
respiratory system
Biology
medicine.disease
Cystic fibrosis
Epithelium
Cystic fibrosis transmembrane conductance regulator
Pathology and Forensic Medicine
Cell biology
Pathogenesis
Mice
medicine.anatomical_structure
medicine
biology.protein
Animals
Humans
Epithelial Physiology
Respiratory epithelium
Inner ear
Subjects
Details
- ISSN :
- 15534014 and 15534006
- Volume :
- 17
- Database :
- OpenAIRE
- Journal :
- Annual Review of Pathology: Mechanisms of Disease
- Accession number :
- edsair.doi.dedup.....df61c5a61653b28c50da4a44010db8ea