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Annual Review of Pathology: Mechanisms of Disease

Volume 17, 2022

Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?

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

Keyword(s): airway, CFTR, cystic fibrosis, epithelial cell, ionocyte, mitochondria-rich cell
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/content/journals/10.1146/annurev-pathol-042420-094031
2022-01-24
2024-04-23
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Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?
Annual Review of Pathology: Mechanisms of Disease 17, 23 (2022); https://doi.org/10.1146/annurev-pathol-042420-094031
/content/journals/10.1146/annurev-pathol-042420-094031
/content/journals/10.1146/annurev-pathol-042420-094031
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  • Article Type: Review Article

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