Our results build upon previous work by our group that indicated endostatin triggers an angiostatic signal cascade propagated by thrombospondin-1 (TSP-1), inhibitor of differentiation/DNA binding-1 (ID1), and bone morphogenetic protein receptor-2 (BMPR2), which have been directly implicated in the pathogenesis of PAH, to inhibit pulmonary endothelial cell migration, proliferation and cell survival [11, 22]. Here, THBS1 is linked to pulmonary arterial hypertension.