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Cell Mol Life Sci. 2017 Jan;74(1):93-115. doi: 10.1007/s00018-016-2391-y. Epub 2016 Oct 6.

Role of CFTR in epithelial physiology.

Author information

1
Epithelial Research Group, Institute for Cell and Molecular Biosciences, University Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.
2
Epithelial Research Group, Institute for Cell and Molecular Biosciences, University Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK. m.a.gray@newcastle.ac.uk.

Abstract

Salt and fluid absorption and secretion are two processes that are fundamental to epithelial function and whole body fluid homeostasis, and as such are tightly regulated in epithelial tissues. The CFTR anion channel plays a major role in regulating both secretion and absorption in a diverse range of epithelial tissues, including the airways, the GI and reproductive tracts, sweat and salivary glands. It is not surprising then that defects in CFTR function are linked to disease, including life-threatening secretory diarrhoeas, such as cholera, as well as the inherited disease, cystic fibrosis (CF), one of the most common life-limiting genetic diseases in Caucasian populations. More recently, CFTR dysfunction has also been implicated in the pathogenesis of acute pancreatitis, chronic obstructive pulmonary disease (COPD), and the hyper-responsiveness in asthma, underscoring its fundamental role in whole body health and disease. CFTR regulates many mechanisms in epithelial physiology, such as maintaining epithelial surface hydration and regulating luminal pH. Indeed, recent studies have identified luminal pH as an important arbiter of epithelial barrier function and innate defence, particularly in the airways and GI tract. In this chapter, we will illustrate the different operational roles of CFTR in epithelial function by describing its characteristics in three different tissues: the airways, the pancreas, and the sweat gland.

KEYWORDS:

Bicarbonate; CFTR; Chloride; Epithelial transport; Physiology

PMID:
27714410
PMCID:
PMC5209439
DOI:
10.1007/s00018-016-2391-y
[Indexed for MEDLINE]
Free PMC Article

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