Molecular pathogenesis of pulmonary arterial hypertension

Recent clinical and experimental studies are redefining the cellular and molecular bases of pulmonary, arterial hypertension (PAH). The genetic abnormalities first identified in association with the idiopathic form of PAH - together with a vast increase in our understanding of cell signaling, cell-transformation, and cell-cell interactions; gene expression; microRNA-processing; and mitochondrial and ion channel function - have helped explain the abnormal response of vascular cells to injury. Experimental and clinical studies now. converge on the intersection and interactions between a genetic predisposition involving the BMPR2 signaling Pathway and an unpaired metabolic and chronic inflammatory state hi the vessel wall The deranged processes' culminate in an exuberant proliferative response that occludes the pulmonary arterial (PA) lumen and obliterates the most distal intraacinar vessels. Here, we describe emerging therapies based on preclinical studies that address these converging pathways.