Additionally, given the previously mentioned unique sensitivity of the lungs to hypoxia and the specific role of HIF-2α in initiating the pathological progression of PAH, numerous small molecule candidates targeting PHD or HIF have been experimentally demonstrated to alleviate PAH (Table 2).27,128,566–570 The concurrent utilization of a tumor suppressor p53 agonist and an HIF-2α antagonist has the potential to specifically and concurrently hinder the hypoxia-triggered proliferation of pulmonary artery smooth muscle cells and the apoptosis of pulmonary artery endothelial cells. Here, EPAS1 is linked to pulmonary arterial hypertension.