Similar to the aforementioned role of ET-1 and its receptors (ETAR and ETBR) in the pathogenesis of PAH, angiotensin II is a potent vasoactive agent that exerts its pleiotropic vasoconstrictor and pro-inflammatory effect by binding to angiotensin receptor type 1 (AT1R). Here, AGTR1 is linked to pulmonary arterial hypertension.