Grant: Operating Grant

Pulmonary hypertension is a lethal disease that often affects young women and results in high blood pressure in the arteries of the lung. Despite all available treatments, it is usually progressive, ultimately resulting in heart failure and death between 3 and 5 after diagnosis. The present research proposal seeks to define the underlying cause of pulmonary hypertension, specifically damage and degeneration of the cells lining the inner surface of blood vessels, called endothelial cells. It is hypothesized that these cells within the fragile lung blood vessels are particularly vulnerable to injury, particularly in patients that have genetic defects known to be associated with this disease. Experiments will be carried out in genetically engineered mouse models of pulmonary hypertension. It is hoped that this work will lead to more effective strategies to prevent and treat this devastating disease, particularly for families carrying the pulmonary hypertension gene.