Protein involved in cystic fibrosis linked to chronic lung diseases

WASHINGTON - A research team at Johns Hopkins Children’s Center has found 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.

The findings pave the way toward new treatments to prevent lung damage caused by infections or cigarette smoke in emphysema.

The protein, called CFTR (cystic fibrosis transmembrane conductance regulator), is already well known for its role in transporting chloride in and out of cells.

In CF, the protein’s chloride-carrying ability is absent due to genetic mutations, resulting in the buildup of thick sticky mucus in the lungs, which causes lung infections and breathing problems.

But the new Hopkins study indicates that CFTR is involved in immune regulation and immune response on a far wider scale.

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.

Thus, the researchers say, by regulating ceramide’s inflammation-causing activity, CFTR appears to be a watchdog for inflammation and cell death.

The study result has been published online in the December issue of The Journal of Immunology. (ANI)