Fig. 6. Effect of MPB-07 on confocal immunofluorescent labelling of CFTR in non-cystic fibrosis and delF508/delF508 cystic fibrosis epithelial cells from nasal brushings. Images show CFTR immunofluorescence in green with the nucleus counterstained with propidium iodide (red). (A,B) Non-cystic fibrosis cells. (C-J) Cystic fibrosis cells. (A,C,E,G,I) Untreated cells. (B,D,F,H,J) Cells treated for 2 hours at 37°C with MPB-07 (250 µM). Results are representative and show the predominant cell type from five non-cystic fibrosis individuals and from four delF508/delF508 cystic fibrosis individuals with or without MPB-07 treatment with the exception of I. This shows an example of delF508-CFTR present throughout the cell, a pattern of distribution present in less than 10% of untreated cells, although in this cystic fibrosis individual, the predominant cell type had a perinuclear delF508-CFTR location similar to that seen in C,E,G. Magnification x1000.
