Alan Harper a.g.s.harper@keele.ac.uk
Islet-intrinsic effects of CFTR mutation
Harper
Authors
Abstract
Cystic fibrosis-related diabetes (CFRD) is the most significant extra-pulmonary comorbidity in cystic fibrosis (CF) patients, and accelerates lung decline. In addition to the traditional view that CFRD is a consequence of fibrotic destruction of the pancreas as a whole, emerging evidence may implicate a role for cystic fibrosis transmembrane-conductance regulator (CFTR) in the regulation of insulin secretion from the pancreatic islet. Impaired first-phase insulin responses and glucose homeostasis have also been reported in CF patients. CFTR expression in both human and mouse beta cells has been confirmed, and recent studies have shown differences in endocrine pancreatic morphology from birth in CF. Recent experimental evidence suggests that functional CFTR channels are required for insulin exocytosis and the regulation of membrane potential in the pancreatic beta cell, which may account for the impairments in insulin secretion observed in many CF patients. These novel insights suggest that the pathogenesis of CFRD is more complicated than originally thought, with implications for diabetes treatment and screening in the CF population. This review summarises recent emerging evidence in support of a primary role for endocrine pancreatic dysfunction in the development of CFRD.
Citation
Harper. (2016). Islet-intrinsic effects of CFTR mutation. Diabetologia, 1350-1355. https://doi.org/10.1007/s00125-016-3936-1
Acceptance Date | Feb 26, 2016 |
---|---|
Publication Date | Mar 31, 2016 |
Journal | Diabetologia |
Print ISSN | 0012-186X |
Publisher | Springer Verlag |
Pages | 1350-1355 |
DOI | https://doi.org/10.1007/s00125-016-3936-1 |
Keywords | beta cells; CFTR; cystic fibrosis; diabetes; endocrine; review |
Publisher URL | https://doi.org/10.1007/s00125-016-3936-1 |
You might also like
Iron oxide nanoparticles as a new tool for treating cardiovascular diseases
(2023)
Conference Proceeding
Platelet Signalling: Calcium
(2017)
Book Chapter
TRP-Na+/Ca2+ Exchanger Coupling
(2016)
Book Chapter
Downloadable Citations
About Keele Repository
Administrator e-mail: research.openaccess@keele.ac.uk
This application uses the following open-source libraries:
SheetJS Community Edition
Apache License Version 2.0 (http://www.apache.org/licenses/)
PDF.js
Apache License Version 2.0 (http://www.apache.org/licenses/)
Font Awesome
SIL OFL 1.1 (http://scripts.sil.org/OFL)
MIT License (http://opensource.org/licenses/mit-license.html)
CC BY 3.0 ( http://creativecommons.org/licenses/by/3.0/)
Powered by Worktribe © 2024
Advanced Search