Vancouver researchers have discovered the cellular pathway that causes lung-damaging inflammation in cystic fibrosis (CF), and that reducing the pathway’s activity also decreases in­flammation. The finding offers a po­tential new drug target for treating CF lung disease, a major cause of illness and death for people with CF.

The discovery was made by a group of Vancouver investigators and trainees based at the Child & Family Research Institute at BC Children’s Hospital, the University of British Columbia, and the Providence Heart + Lung Institute at St. Paul’s Hospital. The research was published online last week in The Journal of Immunology.

For the study, researchers compared the immune response of normal lung cells with that of CF lung cells after exposing both types of cells to bacteria in the lab. In healthy cells, exposure to bacteria triggers the cell to secrete special molecules that attract immune cells to fight the infection. In CF lung cells, the researchers discovered that a series of molecular events called the unfolded protein response is more highly activated. It causes the CF lung cells to secrete more molecules that attract an excessive amount of immune cells, which leads to increased inflammation. They also found that treating the CF cells with a special chemical normalized the unfolded protein response and stabilized the cells’ immune response.

The researchers are planning further study to validate these findings in a larger number of lung cell samples from people with CF.

The study can be viewed at www.jimmunol.org [8].