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The crucial roles of PDGF stimulation and BMP/BMPR2 aberration in PAH pathogenesis. (A) PDGF binds to its receptor tyrosine kinase PDGFR to trigger cell proliferation and migration in PASMCs, which further leads to vascular remodeling. (B) BMP deficiency or BMPR2 mutation results in an impaired antagonistic activity to suppress TGF β1-Stat3-FoxO1 signaling-induced PASMCs proliferation and vascular remodeling.
