Compounds acting on pathogenic pathways of pulmonary arterial hypertension. Compounds acting on pathogenic pathways of pulmonary arterial hypertension. BMPR2: bone morphogenetic peptide receptor 2; VIP: vasoactive intestinal peptide; NO: nitric oxide; RANTES: regulated on activation, normal T-cell expressed and secreted; MCP: monocyte chemoattractant protein; IL: interleukin; PDGF: platelet-derived growth factor; EGF: epidermal growth factor; FGF: fibroblast growth factor; VEGF: vascular epidermal growth factor; PDK: pyruvate dehydrogenase kinase; PDH: pyruvate dehydrogenase; eNOS: endothelial nitric oxide synthase; BH4: tetrahydrobiopterin; ACE: angiotensin-converting enzyme; CCB: calcium channel blocker; HDAC: histone deacetylase; NFATc: nuclear factor of activated T-cells, cytoplasmic; PLC: phospholipase C; BMP: bone morphogenetic peptide; PPAR: peroxisome proliferator-activated receptor; NO2-FA: nitrated fatty acid; IP: prostaglandin receptor; ERA: endothelin receptor antagonist; PDE5-i: phosphodiesterase type-5 inhibitor. Nazzareno Galiè, and Ardeschir-Hossein Ghofrani Eur Respir Rev 2013;22:503-514 ©2013 by European Respiratory Society