Figure 4

Temperature rescue of FAP-CFTR F508del. Rescue of FAP F508del-CFTR by low temperature visualized by confocal fluorescence microscopy is shown. HEK293 cells expressing FAP F508del-CFTR constructs were incubated at the permissive temperature (30°C) for 24 h. Surface expression was confirmed by activation of the impermeant fluorogen (50 nmol/L MG-11p) for both FAP-F508del-CFTR (A) and F508del-CFTR EL4-FAP (C). Cell-permeant staining with 50 nmol/L MG-ester revealed F508del-CFTR at both the cell surface and in intracellular pools (B), and a similar result was found for F508del-CFTR EL4-FAP (D). Controls without low temperature were as shown in Figures 3A and D. The scale bars indicate 10 µm.