Pass the bicarb: the importance of HCO3- for mucin release

Accumulation of thick, sticky mucus is a hallmark of the genetic disease cystic fibrosis (CF) and has a central role in CF pathophysiology. Mutations in the CF transmembrane regulator (CFTR) ion channel are known to result in abnormally thick and sticky mucus; however, why mucus accumulates in CF is...

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Bibliographic Details
Published in:The Journal of clinical investigation 2009-09, Vol.119 (9), p.2535-2537
Main Author: De Lisle, Robert C
Format: Article
Language:English
Subjects:
Animals
Bicarbonates - metabolism
Cystic Fibrosis - etiology
Cystic Fibrosis - genetics
Cystic Fibrosis - physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Cystic Fibrosis Transmembrane Conductance Regulator - physiology
Exocytosis
Humans
Mice
Mice, Inbred CFTR
Models, Biological
Mucins - secretion
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Summary:Accumulation of thick, sticky mucus is a hallmark of the genetic disease cystic fibrosis (CF) and has a central role in CF pathophysiology. Mutations in the CF transmembrane regulator (CFTR) ion channel are known to result in abnormally thick and sticky mucus; however, why mucus accumulates in CF is still not completely understood. In this issue of the JCI, Garcia and colleagues show that mucin--the heavily glycosylated protein contained within mucus--requires CFTR and bicarbonate in order to be released from mouse intestine (see the related article beginning on page 2613). The authors propose a model whereby CFTR-mediated bicarbonate secretion must be concurrent with mucin exocytosis for proper mucin release.
ISSN:0021-9738
1558-8238
DOI:10.1172/JCI40598