Year: 2012
Type: review
Abstract: 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.
DOI: 10.1172/jci60658
Author: Marlene Rabinovitch
Institutions: Stanford Medicine, Stanford University
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4293257851