University of Pittsburgh
Stopping Endothelial Cell Death in Pulmonary Hypertension
Pulmonary hypertension (PH) is a rare but devastating disease characterized by increased pressure of blood vessels in the lungs, which ultimately leads to heart failure. More than 50 percent of the patients die within five years after PH diagnosis. PH is thought to be triggered by an injury to endothelial cells, which line the inside of blood vessels. After an injury, these cells die and do not regenerate, resulting in the loss of small blood vessels in the lungs. We have found that CX3CR1, a molecule known to increase inflammation, is expressed by endothelial cells and can regulate survival of these cells. Endothelial cells in the lungs of patients with PH lose this molecule, leading to cell death. We will study the role of CX3CR1 in stopping endothelial cell death, which can lead to improved treatment for PH.