Using both flow cytometry and optical microscopy, researchers at Johns Hopkins Children's Center have discovered that a protein involved in cystic fibrosis (CF) also regulates inflammation and cell death in emphysema and may be responsible for other chronic lung diseases. Their study indicates that the cystic fibrosis conductance regulator (CFTR) protein, well known for its role in transporting chloride in and out of cells, is involved on a far wider scale in immune regulation and immune response.1
The research—conducted in mice and using lung tissue from people with and without emphysema—shows that those with lung damage from emphysema had less CFTR on the cell surface and that changes in the level of CFTR corresponded directly to disease severity. Decreases in CFTR also corresponded to increased buildup in the lung cells of a fatty molecule called ceramide, a well-known trigger of inflammation and cell death. So by regulating ceramide's inflammation-causing activity, CFTR appears to be a watchdog for inflammation and cell death.
1. M. Bodas et al., J Immunol 186, 602–613 (2011)