Endothelial Dysfunction in Pulmonary Hypertension: Cause or Consequence?

Kurakula, Kondababu and Smolders, Valérie F. E. D. and Tura-Ceide, Olga and Jukema, J. Wouter and Quax, Paul H. A. and Goumans, Marie-José (2021) Endothelial Dysfunction in Pulmonary Hypertension: Cause or Consequence? Biomedicines, 9 (1). p. 57. ISSN 2227-9059

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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.

Item Type: Article
Uncontrolled Keywords: pulmonary hypertension; endothelial dysfunction; vasoactive factors; EndoMT; inflammation; TGF-β; epigenetics
Subjects: STM Repository > Biological Science
Depositing User: Managing Editor
Date Deposited: 07 Mar 2024 08:01
Last Modified: 07 Mar 2024 08:01
URI: http://classical.goforpromo.com/id/eprint/1032

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