EDN1 and pulmonary arterial hypertension: Further, in common with the various forms of PAH [20], the MCT model also displays well-described medial hypertrophy, neointimal proliferation and adventitial changes, EC injury [24,25], increased endothelin-1, downregulation of NO signaling, impaired vasoreactivity and pronounced disruption in BMP and TGF-β signaling linked to increased macrophage recruitment, and inflammation-induced IL-6 expression associated with impaired BMPR2 function [17,26,27].