Uncovering the mechanisms behind pulmonary arterial hypertension, towards a cure

Defining the molecular mechanisms of the OPG-FAS-TRAIL axis in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare but fatal disease. It is most common in young women, who – if untreated – have a prognosis worse than some cancers. PAH is driven by unchecked cell growth in the blood vessels in the lung. This narrows the vessels, raising blood pressure, which puts a dangerous strain on the heart. Current medicines only treat the symptoms of PAH, but researchers are searching for a drug that can halt the disease. Through research funded by the BHF, Dr Allan Lawrie and his team have identified proteins that seem to control the severity of PAH. The first, OPG, circulates in the blood. The second, FAS, is embedded on the surface of lung artery cells and responds to the presence of OPG by stimulating cell growth and proliferation. Dr Lawrie believes that removing OPG from the circulation and, or, blocking FAS in the lung vessels could be key to treating PAH. In this next phase of research, Dr Lawrie will strive to understand how the OPG-FAS system works in the development and progression of PAH. He’ll study the processes in detail in mice, taking us a vital step closer to a cure for this terrible disease.