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, which is a major cause of illness and death for people with CF.
"Developing new drugs that target lung inflammation would be a big step forward," says Dr. Stuart Turvey, who led the research. Dr. Turvey is the director of clinical research and senior clinician scientist at the Child & Family Research Institute and a pediatric immunologist at BC Children's Hospital. He is an associate professor in the Department of Pediatrics at the University of British Columbia.
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.
CF is the most common genetic disease affecting young Canadians. One in every 3600 children born in Canada has CF. There is no cure. A build-up of mucus in the lungs causes people with CF to be susceptible to bacterial lung infections, which trigger inflammation and swelling. Over time, the recurring cycle of infections and inflammation damages the lungs and can lead to the need for lung transplantation. The only treatments for lung inflammation are steroids and anti-inflammatory medications, which can have significant side effects.
The researchers are planning further study to validate these findings in a larger number of lung cell samples from people with CF.
Child & Family Research Institute: http://www.cfri.ca/
This press release was posted to serve as a topic for discussion. Please comment below. We try our best to only post press releases that are associated with peer reviewed scientific literature. Critical discussions of the research are appreciated. If you need help finding a link to the original article, please contact us on twitter or via e-mail.
Bald eagles have made a comeback. See life in the nest now during peak nesting season.
Photos capture a river otter attacking a gator in a Florida river. The otter then feasted, witnesses say.
The 10-meter long Torvosaurus weighed up to five tons.
Researchers say the key to fighting superbugs is individualized treatment plans, and a new nanochip might pave the way
Some farmers have long sworn by mellow tunes to boost Bessie's milk production. The science is hardly conclusive. But a study hints at what might top the barnyard playlist. (Psst: They liked R.E.M.)
Craig Venter, the U.S. scientist who raced the U.S. government to map the human genome over a decade ago and created synthetic life in 2010, is now on a quest to treat age-related disease.
Dr Dave Hone: The Daohugou Fauna is rich in dinosaurs, lizards, pterosaurs, salamanders and mammalsDr Dave Hone
Suppressing a hormone that governs metabolism boosts your chances of living to a grand old age, but there's a downside
Scientists say it is unlikely that any fish can survive in the oceans deeper than about 8,200m.
The discovery of a previously unknown "giant virus" from 30,000 years ago in the Siberian permafrost is bringing researchers closer to understanding the complexity of viruses.