Grant: CIHR Fellowship

Cystic fibrosis (CF) is the most common life-shortening inherited disease in our society. CF is caused by a mutation in the CF transmembrane regulator gene (CFTR), which leads to the accumulation of a thick and sticky mucus in the lungs. CF patients usually have chronic lung inflammation often associated with bacterial infection and continual colonization. Recently, a novel innate defence regulator peptide was identified that suppressed bacterially induced hyperinflammation in CF airway cells and protected mice against bacterially induced lung inflammation. This project will focus on understanding the molecular mechanisms of action of this peptide regulator as an inhibitor of bacterially induced hyperinflammation in CF. Optimization of this peptide sequence will also provide important information for progression to the clinic and a potential treatment for lung inflammation which would significantly improve the quality of life of CF patients.