How a faulty gene leads to pulmonary hypertension

Suppression of IL-6 trans signalling by BMP4: a “missing link” in the development of pulmonary arterial hypertension?

The predicted outcome of many patients with pulmonary arterial hypertension (PAH) is poor because there are very few treatments available. People with PAH have abnormally high blood pressure in the arteries carrying blood to the lungs, causing damage to the right side of the heart. Symptoms include breathlessness or tiredness, and it can result in death from heart failure within a few years. Lung artery walls first become inflamed and then get thicker and narrower. We already know that people who have a fault in the gene that encodes a protein called BMPRII are more likely to develop PAH but we don’t know how this leads to disease. Dr Timothy Palmer and his research team believe that BMPRII limits inflammation in lung arteries by increasing levels of an molecule called SOCS3. They have now been awarded a grant from the BHF to find out how faulty BMPRII reduces SOCS3 levels, and how this leads to inflammation in the lung artery wall and ultimately PAH. They will study the roles of SOCS3 and BMPRII in preventing the narrowing of lung arteries and inflammation in the lung arterial wall. This research may reveal new drug targets for treatment of PAH and could show that drugs, currently used to treat other conditions, such as rheumatoid arthritis, might be useful for PAH.