Fig. 8. Mutant A1038V does not colocalize with cadherin at the plasma membrane but is rescued by VX-809. (A) A1038V ABCA4-GFP (green) stably transfected cells were plated on coverslips. Cells were subsequently fixed and stained with DAPI (blue, nucleus) and antibodies against cadherin (red, plasma membrane). Representative extended focus images are shown. (B) Mutant A1038V shows cell surface expression when treated with VX-809. A1038V ABCA4-GFP (green) stably transfected cells were plated on coverslips. Cells were subsequently fixed and stained with DAPI (blue, nucleus) and antibodies against cadherin (purple, plasma membrane). Colocalization signal is white (white arrows). 3 separate experiments were performed. Representative extended focus images are shown. (C): Pearson's coefficients were obtained for 809 treated and untreated cells. Coefficients were compared using the student's t-test and found to be significant. Conclusion: VX-809 restores surface expression of the mutant A1038V.