Molecular pathogenesis of pulmonary arterial hypertension
Top Cited Papers
- 1 July 2008
- journal article
- review article
- Published by American Society for Clinical Investigation in Journal of Clinical Investigation
- Vol. 118 (7) , 2372-2379
- https://doi.org/10.1172/jci33452
Abstract
Recent investigations have suggested that it might be possible to reverse the pathology of pulmonary arterial hypertension (PAH), a disorder that can be rapidly progressive and fatal despite current treatments including i.v. prostacyclin. This review will address the cellular and molecular processes implicated in clinical, genetic, and experimental studies as underlying the pulmonary vascular abnormalities associated with PAH. Emerging treatments are aimed at inducing apoptosis of abnormal vascular cells that obstruct blood flow and at promoting regeneration of “lost” distal vasculature.Keywords
This publication has 131 references indexed in Scilit:
- Smooth Muscle Protein 22α–Mediated Patchy Deletion of Bmpr1a Impairs Cardiac Contractility but Protects Against Pulmonary Vascular RemodelingCirculation Research, 2008
- Pulmonary arterial remodeling induced by a Th2 immune responseThe Journal of Experimental Medicine, 2008
- Evidence of a Role for Osteoprotegerin in the Pathogenesis of Pulmonary Arterial HypertensionThe American Journal of Pathology, 2008
- The nuclear factor of activated T cells in pulmonary arterial hypertension can be therapeutically targetedProceedings of the National Academy of Sciences, 2007
- Absence of T Cells Confers Increased Pulmonary Arterial Hypertension and Vascular RemodelingAmerican Journal of Respiratory and Critical Care Medicine, 2007
- Alterations of cellular bioenergetics in pulmonary artery endothelial cellsProceedings of the National Academy of Sciences, 2007
- Bone morphogenetic protein-2 upregulates expression and function of voltage-gated K+channels in human pulmonary artery smooth muscle cellsAmerican Journal of Physiology-Lung Cellular and Molecular Physiology, 2006
- HIV-1 Nef Is Associated with Complex Pulmonary Vascular Lesions in SHIV-nef–infected MacaquesAmerican Journal of Respiratory and Critical Care Medicine, 2006
- Increased arginase II and decreased NO synthesis in endothelial cells of patients with pulmonary arterial hypertensionThe FASEB Journal, 2004
- Clinical classification of pulmonary hypertensionPublished by Elsevier ,2004