Pathobiology of pulmonary hypertension - Extracellular matrix

Changes in the extracellular matrix underlie the structural and functional abnormalities in the vessel wall that lead to progressive pulmonary vascula r disease. Studies are reviewed aimed at addressing the cellular and molecu lar programs that regulate the production of the extracellular matrix descr ibing new ways to arrest proliferation and migration of smooth muscle cells and to induce apoptosis. The latter can lead to the reversal of pathology at least in experimental animal models.