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editorial
. 2017 Jan 1;195(1):1–3. doi: 10.1164/rccm.201608-1637ED

Figure 1.

Figure 1.

Current therapies for pulmonary arterial hypertension target prostacyclin, nitric oxide and endothelin-1 signaling pathways. U.S. Food and Drug Administration (FDA)-approved drugs and others in development are shown in the context of the canonical NO, prostacyclin, and endothelin-1 signaling pathways. Reproduced and modified with permission from Reference 2. 6R-BH4 = sapropterin dihydrochloride, tetrahydrobiopterin; AC = adenylyl cyclase; BH2 = dihydrobiopterin; BH4 = tetrahydrobiopterin; cGMP = cyclic guanosine monophosphate; COX = cyclooxygenase; ECEs = endothelin-converting enzymes; eNOS = endothelial NO synthase; ETA = endothelin receptor type A; ETB = endothelin receptor type B; GTP = guanosine-5′-triphosphate; IP = prostaglandin I2 (prostacyclin); PDE5 = phosphodiesterase 5; PGI2 = prostaglandin I2; sGC = soluble guanylyl cyclase.