BMPR2 and pulmonary arterial hypertension: Particularly, in pulmonary arterial hypertension (PAH), as a key DAMP mediator, HMGB1, which is produced and released by RELMα-stimulated ECs, leads to induction of autophagy and inhibition of apoptosis and bone morphogenetic protein receptor 2 (BMPR2) expression in PVSMCs, thus reducing PVSMCs proliferation [53].