title: CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.
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.
authors: Institute for Translational Medicine and 1st Department of Medicine, University of Pécs, Pécs, Hungary. hegyi.peter@med.u-szeged.hu.|MTA-SZTE Translational Gastroenterology Research Group, Szeged, Hungary. hegyi.peter@med.u-szeged.hu.|First Department of Medicine, University of Szeged, Szeged, Hungary. hegyi.peter@med.u-szeged.hu.
authors: Péter
authors: P
authors: Hegyi
authors: Pediatric Gastroenterology Unit, Hadassah Medical Center, Hebrew University, Jerusalem, Israel.
authors: Michael
authors: M
authors: Wilschanski
authors: National Institute of Dental and Craniofacial Research, Bethesda, MD, USA.
authors: Shmuel
authors: S
authors: Muallem
authors: Department of Physiology, McGill University, Montreal, Canada.
authors: Gergely L
authors: GL
authors: Lukacs
authors: Department of Molecular and Cell Biology, Boston University Henry M. Goldman School of Dental Medicine, Boston, MA, USA.
authors: Miklós
authors: M
authors: Sahin-Tóth
authors: Department of Pediatrics, University of Iowa, Carver College of Medicine, Iowa City, IA, USA.
authors: Aliye
authors: A
authors: Uc
authors: Institute for Cell & Molecular Biosciences, University Medical School, Newcastle University, Newcastle upon Tyne, UK.
authors: Michael A
authors: MA
authors: Gray
authors: First Department of Medicine, University of Szeged, Szeged, Hungary.|Department of Pathophysiology, University of Szeged, Szeged, Hungary.
authors: Zoltán
authors: Z
authors: Rakonczay
authors: First Department of Medicine, University of Szeged, Szeged, Hungary.|MTA-SZTE Translational Gastroenterology Research Group, Szeged, Hungary.
authors: József
authors: J
authors: Maléth
mesh_terms: D000818:Animals; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D010195:Pancreatitis / Q000235:genetics / Q000378:metabolism*
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: CFTR; Cystic fibrosis; Epithelial transport; Pancreas; Pancreatitis