New research from OMRF could help doctors activate some natural defenses that are turned off in patients with cystic fibrosis.
Working with researchers from Case Western Reserve University and Emory University, OMRF scientist Scott Plafker, Ph.D., R.Ph., found that malfunctions in a pair of proteins are to blame for inflammation in cystic fibrosis.
Cystic fibrosis is an inflammatory disease that affects the lungs and digestive system which affects about 30,000 Americans and about 70,000 patients worldwide. Caused by a defective gene, the disease forces the body to produce thick, sticky mucus that clogs the lungs, making it hard to breathe. Patients with cystic fibrosis have a reduced lifespan and often die in their 30s or 40s.
Using an antibody designed by Plafker, the group was able to trace inflammation in the lungs to a pair of proteins.
“The proteins work at cross-purposes,” said Plafker, who joined OMRF in 2011. “One is there to protect from inflammation and the other causes it. Normally, there’s a give and take, but in patients with cystic fibrosis, the pro-inflammatory protein dominates the competition.”
The research could lead to a new treatment for cystic fibrosis, helping to extend the lives of patients, he said.
The next step for scientists is to learn if increasing the anti-inflammatory protein, either by encouraging the body to make more or administering a lab-grown protein, will reduce inflammation.
The research was published in The American Journal of Physiology – Lung Cellular and Molecular Physiology.