Molecular pathogenesis of pulmonary arterial hypertension

  • Rabinovitch M
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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 impaired metabolic and chronic inflammatory state in the vessel wall. These 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.

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  • Marlene Rabinovitch

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