CFTR: a missing link between exocrine and endocrine pancreas?

Tzyh-Chang Hwang

doi:10.1007/s11427-014-4740-0

Abstract

CFTR: a missing link between exocrine and endocrine pancreas?

Highlights

Cystic fibrosis (CF), a life-shortening hereditary disease mainly afflicting people of Caucasian origins, is caused by loss-of-function mutations in the CFTR (Cystic Fibrosis Transmembrane conductance Regulator) gene, which encodes a phosphorylation-activated, but ATP-gated anion channel expressed primarily in epithelial cells
CFTR serves as a major pathway for the secretion of bicarbonate in the pancreas, and dysfunction of the CFTR channel suffices to account for exocrine pancreatic abnormities in patients with CF
Cystic fibrosis-related diabetes (CFRD), a comorbidity found in ~50% of adult CF patients, is an endocrine disorder that gravely affects the clinical outcomes of CF

Summary

Introduction

Cystic fibrosis (CF), a life-shortening hereditary disease mainly afflicting people of Caucasian origins, is caused by loss-of-function mutations in the CFTR (Cystic Fibrosis Transmembrane conductance Regulator) gene, which encodes a phosphorylation-activated, but ATP-gated anion channel expressed primarily in epithelial cells. In a host of exocrine tissues, activation of CFTR in the apical membrane of epithelial cells establishes an electric potential that drives the transepithelial movement of a counter ion such as sodium.

Results

Conclusion