Consistently, PAH development is associated with vasoconstriction, increased ECM production, EndMT, upregulation of inflammatory modulators, immune cell infiltration and extensive EC, SMC and pericyte migration and proliferation that is mediated by BMP-TGFβ signaling, as well as growth factors such as PDGF and FGF [70]. Here, TGFB1 is linked to pulmonary arterial hypertension.