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CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis

Lookup NU author(s): Dr Michael Gray, Dr Zoltan Rakonczay

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Abstract

Cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel that conducts chloride and bicarbonate ions across epithelial cell membranes. Mutations in the CFTR gene diminish the ion channel function and lead to impaired epithelial fluid transport in multiple organs such as the lung and the pancreas resulting in cystic fibrosis. Heterozygous carriers of CFTR mutations do not develop cystic fibrosis but exhibit increased risk for pancreatitis and associated pancreatic damage characterized by elevated mucus levels, fibrosis, and cyst formation. Importantly, recent studies demonstrated that pancreatitis causing insults, such as alcohol, smoking, or bile acids, strongly inhibit CFTR function. Furthermore, human studies showed reduced levels of CFTR expression and function in all forms of pancreatitis. These findings indicate that impairment of CFTR is critical in the development of pancreatitis; therefore, correcting CFTR function could be the first specific therapy in pancreatitis. In this review, we summarize recent advances in the field and discuss new possibilities for the treatment of pancreatitis.


Publication metadata

Author(s): Hegyi P, Wilschanski M, Muallem S, Lukacs GL, Sahin-Toth M, Uc A, Gray MA, Rakonczay Z, Maleth J

Publication type: Review

Publication status: Published

Journal: Reviews of Physiology, Biochemistry and Pharmacology Vol. 170

Year: 2016

Volume: 170

Pages: 37-66

Online publication date: 09/02/2016

Acceptance date: 01/01/1900

ISSN (print): 0303-4240

Publisher: SPRINGER INT PUBLISHING AG

URL: http://dx.doi.org/10.1007/112_2015_5002

DOI: 10.1007/112_2015_5002


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