Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.

ABSTRACT: Efficient trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) to and from the cell surface is essential for maintaining channel density at the plasma membrane (PM) and ensuring proper physiological activity. The most common mutation, F508del, exhibits reduced surface expression and impaired function despite treatment with currently available pharmacological small molecules, called correctors. To gain more detailed insight into whether CFTR enters compartments that allow corrector stabilization in the cell periphery, we investigated the peripheral trafficking itineraries and kinetics of wild type (WT) and F508del in living cells using high-speed fluorescence microscopy together with fluorogen activating protein detection. We directly visualized internalization and

SUBMITTER: Holleran JP 

PROVIDER: S-EPMC3687701 | biostudies-literature | 2013 Jun

REPOSITORIES: biostudies-literature