The cellular pathway that causes lung-damaging inflammation in cystic fibrosis (CF) has not only been identified, but scientists have also discovered that decreasing the pathway’s activity can cause the inflammation to reduce.
A previous report indicated that researchers were able to prevent cystic fibrosis lung disease in a mouse model by spraying amiloride into the animal’s lungs.
The current finding came from Vancouver experts who published the findings in the Journal of Immunology and believe that their research can aid to the development of a new drug target for the treatment of CF lung disease. This is significant, because many CF patients develop this illness and experience death because of it.
“Developing new drugs that target lung inflammation would be a big step forward,” said research leader Dr. Stuart Turvey, director of clinical research, senior clinician scientist at the Child & Family Research Institute and a pediatric immunologist at BC Children’s Hospital.
The team analyzed the difference between normal lung cells’ immune response with the immune response of CF lung cells, after exposing both to bacteria.
When healthy cells are exposed to bacteria, it causes the cell to secrete special molecules that pull immune cells to protect against infection.
The unfolded protein response, which is a succession of molecular events, is more highly activated when the CF lung cells are exposed to bacteria, according to the authors. This results in the secretion of more molecules that attract a significant amount of immune cells, eventually leading to more inflammation.
When using a special chemical to treat the CF cells, the unfolded protein response went back to normal and the cells’ immune response became stable.
Cystic Fibrosis is the most prevalent genetic disease in Canadian youths. In Canada, one in every 3,600 kids are born with CF. Unfortunately, there is no treatment to cure the disease.
Individuals with CF become vulnerable to bacterial lung infections when there is a build-up of mucus in the lungs, which causes inflammation and swelling. Since infections are constantly developing, over time, the lungs become extremely damaged and may lead to the need for a transplant.
Lung inflammation can currently only be treated with steroids and anti-inflammatory drugs, which have serious negative effects.
The authors plan to conduct more research with a larger number of lung cell samples from CF patients in order to confirm their findings.
Written by Sarah Glynn