ABSTRACT
Although many factors act in concert to orchestrate the pulmonary vascular remodeling that characterizes pulmonary arterial hypertension (PAH), recent advances in our understanding of the cellular and molecular mechanisms driving these pathologic changes have demonstrated that alterations in certain key pathways play a central role in either the initiation or progression of disease. Changes in these pathways, involving alterations in both intracellular signaling and extracellular cues, influence the function of a variety of cell types, including pulmonary vascular endothelial and smooth muscle cells, adventitial fibroblasts, cardiomyocytes, and a variety of immune cell subsets.
