Defective CFTR leads to aberrant β-catenin activation and kidney fibrosis.

ABSTRACT: Cystic fibrosis transmembrane conductance regulator (CFTR), known as a cAMP-activated Cl^- channel, is widely expressed at the apical membrane of epithelial cells in a wide variety of tissues. Of note, despite the abundant expression of CFTR in mammalian kidney, the role of CFTR in kidney disease development is unclear. Here, we report that CFTR expression is downregulated in the UUO (unilateral ureteral obstruction)-induced kidney fibrosis mouse model and human fibrotic kidneys. Dysfunction or downregulation of CFTR in renal epithelial cells leads to alteration of genes involved in Epithelial-Mesenchymal Transition (EMT) and kidney fibrosis. In addition, dysregulation of CFTR activates canonical Wnt/β-catenin signaling pathways, whereas the β-catenin inhibitor reverses the effec

SUBMITTER: Zhang JT 

PROVIDER: S-EPMC5507915 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature