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Front. Physiol. | doi: 10.3389/fphys.2018.01585

Cystic fibrosis of the pancreas: the role of altered Ca2+ signalling and mitochondrial function in exocrine cell damage

  • 1HAS-USZ Momentum Epithel Cell Signalling and Secretion Research Group, Hungary
  • 2First Department of Medicine, University of Szeged, Hungary
  • 3Department of Public Health, University of Szeged, Hungary

Cystic fibrosis is the most common genetic disorder that cause a significant damage in secretory epithelial cells due to impaired ion secretion by the cystic fibrosis tarnsmembrane conductance regulator (CFTR) Cl- channel. The organs most frequently damaged by the disease are the lung and the pancreas. The damage of the exocrine pancreas leads to pancreatic insufficiency, abdominal pain and an increased risk of acute pancreatitis in cystic fibrosis patients causing significant decrease in the quality of life. Therefore the understanding of the role of CFTR in the ductal secretory functions is highly interesting. However, several recent study indicated that CFTR has a central role in the intracellular signalling processes and is now considered as a signalling hub in epithelial cells. In addition, impaired CFTR expression has been connected with sustained elevation of the intracellular Ca2+ and with damaged mitochondrial function in epithelial cells. These observations are obscured in the exocrine pancreas, although very similar intracellular changes were described in acute pancreatitis. Therefore in this review we would like to summerize the complex role of CFTR in the exocrine pancreas with a special focus on the intracellar signalling and mitochondrial function.

Keywords: CFTR, cystic fibrosis transmembrane conductance regulator, Epithelial Cells, Ca signaling, mitochdrial damage, cystic fibrosis, Exocrine pancreas

Received: 31 Jul 2018; Accepted: 23 Oct 2018.

Edited by:

Michael Chvanov, University of Liverpool, United Kingdom

Reviewed by:

Savio George Barreto, Medanta The Medicity, India
Olga A. Mareninova, University of California, Los Angeles, United States  

Copyright: © 2018 Maléth, Madacsy and Pallagi. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Dr. József Maléth, HAS-USZ Momentum Epithel Cell Signalling and Secretion Research Group, Szeged, Hungary,