Role of epithelial HCO3⁻ transport in mucin secretion: lessons from cystic fibrosis

The invitation to present the 2010 Hans Ussing lecture for the Epithelial Transport Group of the American Physiological Society offered me a unique, special, and very surprising opportunity to join in saluting a man whom I met only once, but whose work was the basis, not only for my career, but also...

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Bibliographic Details
Published in:American Journal of Physiology: Cell Physiology 2010-12, Vol.299 (6), p.C1222-C1233
Main Author: Quinton, Paul M
Format: Article
Language:English
Subjects:
Animals
Bicarbonates - metabolism
Cystic Fibrosis - genetics
Cystic Fibrosis - metabolism
Cystic Fibrosis - pathology
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Cystic Fibrosis Transmembrane Conductance Regulator - physiology
Female
Humans
Ion Transport - genetics
Ion Transport - physiology
Lung - pathology
Lung - physiopathology
Male
Mice
Mucins - secretion
Mucus - metabolism
Mucus - secretion
Reviews
Sodium Chloride - metabolism
Sweat Glands - metabolism
Sweat Glands - pathology
Sweat Glands - physiopathology
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Summary:The invitation to present the 2010 Hans Ussing lecture for the Epithelial Transport Group of the American Physiological Society offered me a unique, special, and very surprising opportunity to join in saluting a man whom I met only once, but whose work was the basis, not only for my career, but also for finding the molecular defect in the inherited disease cystic fibrosis (CF). In this context, I will venture to make the tribute with a new explanation of why a mutation in a single gene that codes for an anion channel can cause devastation of multiple epithelial systems with pathogenic mucus. In so doing, I hope to raise awareness of a new role for that peculiar anion around which so much physiology revolves, HCO(3)(-). I begin by introducing CF pathology as I question the name of the disease as well as the prevalent view of the basis of its pathology by considering: 1) mucus, 2) salt, and 3) HCO(3)(-). I then present recent data showing that HCO(3)(-) is required for normal mucus discharge, and I will close with conjecture as to how HCO(3)(-) may support mucus discharge and why the failure to transport this electrolyte is pathogenic in CF.
ISSN:0363-6143
1522-1563
DOI:10.1152/ajpcell.00362.2010