Cellular and molecular pathobiology of pulmonary arterial hypertension.
By M. Humbert, N. Morrell, S. Archer, K. Stenmark, M. MacLean, I. Lang, B. Christman, E. Weir, Oliver Eickelberg, N. Voelkel and 1 more. Journal of the American College of Cardiology, 2004
1,633 citations recorded by the source corpus.
Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction, remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary vascular resistance in PAH. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment of the pulmonary arterial wall. Indeed, each cell type (endothelial, smooth muscle, and fibroblast), as well as inflammatory cells and platelets, may play a significant role in PAH. Pulmonary vasoconstriction is believed to be an early component of the pulmonary hypertensive process. Excessive vasoconstriction has been related to abnormal function or expression of potassium channels and to endothelial dysfunction. Endothelial dysfunction leads to chronically impaired production of vasodilators such as nitric oxide and prostacyclin along with overexpression of vasoconstrictors such as endothelin (ET)-1. Many of these abnormalities not only elevate vascular tone and promote vascular remodeling but also represent logical pharmacological targets. Recent genetic and pathophysiologic studies have emphasized the relevance of several mediators in this condition, including prostacyclin, nitric oxide, ET-1, angiopoietin-1, serotonin, cytokines, chemokines, and members of the transforming-growth-factor-beta superfamily. Disordered proteolysis of the extracellular matrix is also evident in PAH. Future studies are required to find which if any of these abnormalities initiates PAH and which ones are best targeted to cure the disease.
Subfields: Cardiology and Cardiovascular Medicine, Genetics, Pulmonary and Respiratory Medicine
View Paper: https://www.semanticscholar.org/paper/61afeb87733287cb7985f7fdc60cf9c88654614f
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In Pulmonary and Respiratory Medicine
Citations in the corpus
- Pulmonary arterial hypertension (2013, background): “(NO) and prostacyclin and an increased production of vasoconstrictors such as endothelin-1 [153].”
- Animal models of pulmonary arterial hypertension: the hope for etiological discovery and pharmacological cure. (2009, background): “The pathogenesis of increased precapillary pulmonary vascular resistance in PAH is generally ascribed to the combined effects of sustained vasoconstriction, arterial wall remodeling, and in situ thrombosis (23, 69, 133, 188).”
- Tadalafil Therapy for Pulmonary Arterial Hypertension (2009, neutral): “Anorexigen use 2 (2) 5 (6) 1 (1) 4 (5) 4 (5)”