Figure 3

Correctors rescue the trafficking defect of FAP-CFTR F508del. HEK293 cells stably expressing FAP-F508del-CFTR (A-C) or F508del-CFTR EL4-FAP (D-F) were imaged using confocal fluorescence microscopy. After treatment with the vehicle (DMSO) control for 24 h, there was no staining observed in the presence of cell-impermeant fluorogen, 50 nmol/L MG-11p (A, D). A strong intracellular signal that was not localized to the plasma membrane was revealed on addition of the cell-permeant fluorogen, MG-ester (B, E). After treatment with a combination of correctors (C4 and C18 for 24 h), F508del-CFTR was detected at the cell surface using cell-impermeant fluorogen, 50 nmol/L MG-11p (C, F). The scale bars indicate 10 µm.