MicroRNA-9 downregulates the ANO1 chloride channel and contributes to cystic fibrosis lung pathology.

ABSTRACT: Cystic fibrosis results from reduced cystic fibrosis transmembrane conductance regulator protein activity leading to defective epithelial ion transport. Ca²⁺-activated Cl^- channels mediate physiological functions independently of cystic fibrosis transmembrane conductance regulator. Anoctamin 1 (ANO1/TMEM16A) was identified as the major Ca²⁺-activated Cl^- channel in airway epithelial cells, and we recently demonstrated that downregulation of the anoctamin 1 channel in cystic fibrosis patients contributes to disease severity via an unknown mechanism. Here we show that microRNA-9 (miR-9) contributes to cystic fibrosis and downregulates anoctamin 1 by directly targeting its 3'UTR. We present a potential therapy based on blockage of miR-9 binding to t

SUBMITTER: Sonneville F 

PROVIDER: S-EPMC5617894 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature