CFTR: A new horizon in the pathomechanism and treatment of pancreatitis

P. Hegyi, Michael Wilschanski, Shmuel Muallem, Gergely L. Lukacs, Miklós Sahin-Tóth, Aliye Uc, Michael A. Gray, Z. Rakonczay, József Maléth

Research output: Contribution to journalArticle

19 Citations (Scopus)

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.

Original languageEnglish
JournalReviews of Physiology, Biochemistry and Pharmacology
Volume170
DOIs
Publication statusPublished - 2016

Fingerprint

Cystic Fibrosis Transmembrane Conductance Regulator
Pancreatitis
Cystic Fibrosis
Mutation
Chloride Channels
Mucus
Cell membranes
Regulator Genes
Bicarbonates
Bile Acids and Salts
Ion Channels
Cysts
Pancreas
Fibrosis
Genes
Smoking
Epithelial Cells
Alcohols
Cell Membrane
Lung

Keywords

  • CFTR
  • Cystic fibrosis
  • Epithelial transport
  • Pancreas
  • Pancreatitis

ASJC Scopus subject areas

  • Pharmacology

Cite this

CFTR : A new horizon in the pathomechanism and treatment of pancreatitis. / Hegyi, P.; Wilschanski, Michael; Muallem, Shmuel; Lukacs, Gergely L.; Sahin-Tóth, Miklós; Uc, Aliye; Gray, Michael A.; Rakonczay, Z.; Maléth, József.

In: Reviews of Physiology, Biochemistry and Pharmacology, Vol. 170, 2016.

Research output: Contribution to journalArticle

Hegyi, P. ; Wilschanski, Michael ; Muallem, Shmuel ; Lukacs, Gergely L. ; Sahin-Tóth, Miklós ; Uc, Aliye ; Gray, Michael A. ; Rakonczay, Z. ; Maléth, József. / CFTR : A new horizon in the pathomechanism and treatment of pancreatitis. In: Reviews of Physiology, Biochemistry and Pharmacology. 2016 ; Vol. 170.
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