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- W3118583265 abstract "Pulmonary arterial hypertension (PAH) is a rare, complex, and progressive disease that is characterized by the abnormal remodeling of the pulmonary arteries that leads to right ventricular failure and death. Although our understanding of the causes for abnormal vascular remodeling in PAH is limited, accumulating evidence indicates that endothelial cell (EC) dysfunction is one of the first triggers initiating this process. EC dysfunction leads to the activation of several cellular signalling pathways in the endothelium, resulting in the uncontrolled proliferation of ECs, pulmonary artery smooth muscle cells, and fibroblasts, and eventually leads to vascular remodelling and the occlusion of the pulmonary blood vessels. Other factors that are related to EC dysfunction in PAH are an increase in endothelial to mesenchymal transition, inflammation, apoptosis, and thrombus formation. In this review, we outline the latest advances on the role of EC dysfunction in PAH and other forms of pulmonary hypertension. We also elaborate on the molecular signals that orchestrate EC dysfunction in PAH. Understanding the role and mechanisms of EC dysfunction will unravel the therapeutic potential of targeting this process in PAH." @default.
- W3118583265 created "2021-01-18" @default.
- W3118583265 creator A5000932307 @default.
- W3118583265 creator A5005451442 @default.
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- W3118583265 creator A5015411763 @default.
- W3118583265 creator A5056821715 @default.
- W3118583265 creator A5062703041 @default.
- W3118583265 date "2021-01-09" @default.
- W3118583265 modified "2023-10-15" @default.
- W3118583265 title "Endothelial Dysfunction in Pulmonary Hypertension: Cause or Consequence?" @default.
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