Endothelial cells in the pathogenesis of pulmonary arterial hypertension.
Endothelial cells in the pathogenesis of pulmonary arterial hypertension.
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DOI：
10.1183/13993003.03957-2020
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发表时间：
2021-09
期刊：
影响因子：
--
通讯作者：
Zhao YY
中科院分区：
文献类型：
--
作者：
Evans CE;Cober ND;Dai Z;Stewart DJ;Zhao YY
Pulmonary arterial hypertension (PAH) is a devastating disease that involves pulmonary vasoconstriction, small vessel obliteration, large vessel thickening and obstruction, and development of plexiform lesions. PAH vasculopathy leads to progressive increases in pulmonary vascular resistance, right heart failure, and ultimately, premature death. Besides other cell types that are known to be involved in PAH pathogenesis (e.g. smooth muscle cells, fibroblasts, and leukocytes), recent studies demonstrate a crucial role of endothelial cells (ECs) in the initiation and progression of PAH. The EC-specific role in PAH is multi-faceted and impacts upon numerous pathophysiological processes including vasoconstriction, inflammation, coagulation, metabolism, and oxidative/nitrative stress, as well as cell viability, growth, and differentiation. In this review, we describe how EC dysfunction and cell signaling regulate the pathogenesis of PAH. We also highlight areas of research that warrant attention in future studies, and discuss potential molecular signaling pathways in ECs that could be targeted therapeutically in the prevention and treatment of PAH.
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