Vascular repair and regeneration as a therapeutic target for pulmonary arterial hypertension.

ABSTRACT: The last decade has seen substantial changes in our understanding of the pathobiology of pulmonary arterial hypertension (PAH), a severe and devastating disease without curative treatment. It is now accepted that injury to the endothelial cells of the pulmonary arteries is central for the subsequent development of lumen-obliterative lung vascular lesions. A variety of circulating and lung-resident progenitor and stem cells likely contribute to vascular integrity, and evidence for the presence of cells expressing stem and progenitor cell markers is found inside and in the immediate vicinity of pulmonary vascular lesions in PAH. The currently available vasodilator therapies mainly target enhanced vasoconstriction in the lung circulation and help to maintain or improve right ventricular funct

SUBMITTER: Farkas L 

PROVIDER: S-EPMC3688480 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature