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

Péter Hegyi; Michael Wilschanski; Shmuel Muallem; Gergely L Lukacs; Miklós Sahin-Tóth; Aliye Uc; Michael A Gray; Zoltán Rakonczay Jr; József Maléth

doi:10.1007/112_2015_5002

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

Journal article

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

Péter Hegyi, Michael Wilschanski, Shmuel Muallem, Gergely L Lukacs, Miklós Sahin-Tóth, Aliye Uc, Michael A Gray, Zoltán Rakonczay Jr and József Maléth

Reviews of physiology, biochemistry and pharmacology, Vol.170, pp.37-66

2016

DOI: 10.1007/112_2015_5002

PMCID: PMC5232416

PMID: 26856995

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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.

Pancreatitis - genetics

Cystic Fibrosis Transmembrane Conductance Regulator - genetics

Cystic Fibrosis Transmembrane Conductance Regulator - metabolism

Animals

Pancreatitis - metabolism

Humans

Details

Title: Subtitle: CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis
Creators: Péter Hegyi - First Department of Medicine, University of Szeged, Szeged, Hungary. hegyi.peter@med.u-szeged.hu
Michael Wilschanski - Pediatric Gastroenterology Unit, Hadassah Medical Center, Hebrew University, Jerusalem, Israel
Shmuel Muallem - National Institute of Dental and Craniofacial Research, Bethesda, MD, USA
Gergely L Lukacs - Department of Physiology, McGill University, Montreal, Canada
Miklós Sahin-Tóth - Department of Molecular and Cell Biology, Boston University Henry M. Goldman School of Dental Medicine, Boston, MA, USA
Aliye Uc - Department of Pediatrics, University of Iowa, Carver College of Medicine, Iowa City, IA, USA
Michael A Gray - Institute for Cell & Molecular Biosciences, University Medical School, Newcastle University, Newcastle upon Tyne, UK
Zoltán Rakonczay Jr - Department of Pathophysiology, University of Szeged, Szeged, Hungary
József Maléth - MTA-SZTE Translational Gastroenterology Research Group, Szeged, Hungary
Resource Type: Journal article
Publication Details: Reviews of physiology, biochemistry and pharmacology, Vol.170, pp.37-66
DOI: 10.1007/112_2015_5002
PMID: 26856995
PMCID: PMC5232416
NLM abbreviation: Rev Physiol Biochem Pharmacol
eISBN: 9783319314921; 3319314920
ISSN: 0303-4240
eISSN: 1617-5786
Publisher: Germany
Grant note: ZIA DE000735 / NIDCR NIH HHS R24 DK096518 / NIDDK NIH HHS R21 DK096327 / NIDDK NIH HHS U01 DK108334 / NIDDK NIH HHS R01 DK097820 / NIDDK NIH HHS R01 DK058088 / NIDDK NIH HHS R01 DK075302 / NIDDK NIH HHS P30 DK054759 / NIDDK NIH HHS
Language: English
Date published: 2016
Academic Unit: Stead Family Department of Pediatrics; Radiation Oncology; Gastroenterology, Hepatology, Pancreatology, and Nutrition
Record Identifier: 9984047622002771

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