Figure 1.

Current treatment approaches and novel targets in pulmonary arterial hypertension. Current treatment approaches focus on endothelin, nitric oxide, and prostacyclin signaling. Novel treatment targets address 1) epigenetic mechanisms/transcriptional factors; 2) oxidative stress; 3) hypoxia and metabolic signaling; 4) BMPR2–mediated signaling; 5) tyrosine kinase and growth factor signaling; 6) fibrosis and extracellular matrix remodeling; and 7) inflammation and immune cell infiltration. 4PBA = 4-phenylbutyric acid; AA = arachidonic acid; ActRII = activin receptor type II; BMP = bone morphogenetic protein; BMPR2 = bone morphogenetic protein receptor type 2; cDC = conventional dendritic cell; DNMT3B = DNA methyltransferase 3 β; ET = endothelin; GDFs = growth and differentiation factors; HIF = hypoxia-inducible factor; JAGGED-1 = jagged canonical Notch ligand 1; JAK = Janus kinase; MAO-A = monoaminoxidase A; MMP = matrix metalloproteinase; NUDT1 = nudix hydrolase 1; NEDD9 = neural precursor cell–expressed developmentally downregulated protein 9; NFU1 = NFU1 iron-sulfur cluster scaffold; NO = nitric oxide; NOS = nitric oxide synthase; PAEC = pulmonary artery endothelial cell; PASMC = pulmonary arterial smooth muscle cell; PDE5 = phosphodiesterase 5; PGI2 = prostacyclin; PINK1 = phosphatase and tensin homolog–induced kinase 1; SDF1 = stromal cell–derived factor 1; sGC = soluble guanylate cyclase; SOX9 = SRY-box transcription factor 9; SOX17 = SRY-box transcription factor 17; SPARC = secreted protein acidic and rich in cysteine; TBX4 = T-box transcription factor 4; TET2 = Tet methylcytosine dioxygenase 2; TWIST1 = Twist family BHLH transcription factor 1; TYKRIL = tyrosine kinase receptor–inducing long noncoding RNA.