Localisation of wild-type and DeltaF508-CFTR in nasal epithelial cells

Pflugers Arch. 2001:443 Suppl 1:S117-20. doi: 10.1007/s004240100657. Epub 2001 Jul 13.


Wild-type and the DeltaF508 mutation of the cystic fibrosis transmembrane conductance regulator (DeltaF508-CFTR) were localised by confocal imaging in DeltaF508/DeltaF508 native airway epithelial cells using a well-characterised CFTR antibody. Surface nasal epithelial cells from three control and three CF individuals were obtained from nasal brushings. Cells were fixed, permeabilised and incubated with first antibody for 18 h at 4 degrees C. Following labelling with second antibody, cells were viewed with the confocal microscope. Wild-type CFTR was localised predominantly apically, whereas DeltaF508-CFTR was located mainly inside the cell in a region close to the nucleus. Incubation of cells with MPB-07 (250 microM) at 37 degrees C for 2 h resulted in pronounced movement of DeltaF508-CFTR to the cell periphery, but did not change the localisation of wild-type CFTR. The results show that DeltaF508-CFTR is mislocalised in native nasal epithelial cells and that its distribution is altered in response to the new CFTR activator, MPB-07. The findings should lead to development of a rational drug treatment for CF patients carrying the DeltaF508 mutation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibody Specificity
  • Cystic Fibrosis / metabolism
  • Cystic Fibrosis Transmembrane Conductance Regulator / analysis*
  • Cystic Fibrosis Transmembrane Conductance Regulator / immunology
  • Epithelial Cells / chemistry*
  • Humans
  • Microscopy, Confocal
  • Nasal Mucosa / chemistry*
  • Nasal Mucosa / cytology


  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator