Modelling cardiomyocyte biology in pulmonary arterial hypertension using induced pluripotent stem cells
Pulmonary arterial hypertension (PAH) is characterized by increased blood pressure in the blood vessels of the lung which ultimately results in heart failure and death. In particular, patients with mutations in a gene called BMPR2 appear more susceptible to death from right heart failure. The mechanism underlying this is unknown and there is currently no easy way to study this in humans. Induced pluripotent stem cell (iPSC), which can be generated from almost anyone and have the capacity to grow into any tissue on the body, were used to grow heart cells in the lab from patients with BMPR2 mutations. In collaboration with Nottingham University we used these cells to uncover important new insights into how hearts cells in patients with PAH change during the disease process. This work is poised for expansion, including screening for new medicines to treat cardiac hypotrophy, leading to real tangible benefits for patients.