Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.

ABSTRACT:

Background

The clinical response to cystic fibrosis transmembrane conductance regulator (CFTR) modulators varies between people with cystic fibrosis (CF) of the same genotype, in part through the action of solute carriers encoded by modifier genes. Here, we investigate whether phosphate transport by SLC34A2 modulates the function of F508del-CFTR after its rescue by CFTR correctors.

Methods

With Fischer rat thyroid (FRT) cells heterologously expressing wild-type and F508del-CFTR and fully-differentiated CF and non-CF human airway epithelial cells, we studied SLC34A2 expression and the effects of phosphate on CFTR-mediated transepithelial ion transport. F508del-CFTR was trafficked to the plasma membrane by incubation with different CFTR correctors (alone or in combination) or

SUBMITTER: Saint-Criq V 

PROVIDER: S-EPMC8503924 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature