Grant: Operating Grant

Idiopathic pulmonary fibrosis (IPF) is a devastating disease since there is no cure or effective therapy. In North America and Canada there are over 200,000 patients with this disease. Of these more than 40,000 die annually. Currently we think that the pathology of IPF follows a pathway similar to normal wound healing, although there is progression of the disease because the normal "braking" mechanisms do not function properly. As a consequence, too much connective tissue is produced in the lungs and the ability to transfer oxygen to the bloodstream is severely impaired. My research focuses on the cells that produce the connective tissue, called myofibroblasts. We have identified a protein that we think regulates how myofibroblasts appear in the lung and produce connective tissue but do not die. By reducing the activation of this protein, the hope is that we can make myofibroblasts function normally.