Endothelial Bone Morphogenetic Protein Signaling in Pulmonary Arterial Hypertension

Pulmonary hypertension (PH), high blood pressure in the pulmonary circulation, can occur in the context of common lung and cardiovascular conditions. Pulmonary arterial hypertension (PAH), a rarer condition with a poor prognosis, is a subgroup of PH, defined by pathology and responsiveness to existing licensed drugs. Human genetic studies have revealed causal mutations in genes encoding bone morphogenetic protein receptor type II ( BMPR2 ) and other components of BMP signaling pathways. This review will provide an overview of PAH pathophysiology, the genetic and cellular evidence on the critical role of endothelial BMP signaling in PAH, including BMP signaling complex regulation, and potential approaches to target these signaling complexes for treating PAH.