Grant: Project Grant

Pulmonary fibrosis occurs when the lung becomes scarred and stiffened, leading to death from respiratory failure. The average survival of an individual with pulmonary fibrosis is only 3-5 years, worse than many types of cancer. One of the reasons is that it is very hard to diagnose pulmonary fibrosis. Furthermore, there is no biomarker that tells if you have pulmonary fibrosis. Although the cause of pulmonary fibrosis is not known, risk factors include genetic susceptibility and cigarette smoke. There is no cure for pulmonary fibrosis and remarkably few effective therapies. This research will investigate a novel protein- called human antigen R (HuR)- that has never been studied in pulmonary fibrosis. However, HuR may control protein expression associated with pulmonary fibrosis that leads to respiratory failure. Based on what we know about HuR in cancer, we predict that HuR will promote pulmonary fibrosis when it does not behave in a normal fashion. Some things that cause HuR to behave improperly include cigarette smoke and radiation- risk factors for pulmonary fibrosis. The localization of HuR within a cell shows that someone with cancer will progress very rapidly. It is not known where HuR is found in the lung in fibrosis conditions, but we predict that in a person with pulmonary fibrosis, HuR levels and location in the lung may be very different compared to someone without the disease. Determining this may be one way to tell who has the disease and who does not. Finally, inhibiting HuR with a drug would be one way to prevent its function from being irregular. Using animal models of fibrosis, biological samples from individuals with IPF as well as cell and molecular biology techniques, we will test the therapeutic and prognostic utility of HuR in pulmonary fibrosis. In essence, HuR represents an unexplored and innovative pathway that will ultimately serving as a catalyst for future experiments to explore novel drugs and the utility of HuR as a biomarker.