Journal
REVUE DES MALADIES RESPIRATOIRES
Volume 40, Issue 3, Pages 234-238Publisher
MASSON EDITEUR
DOI: 10.1016/j.rmr.2023.01.017
Keywords
Pulmonary vascular remodeling; Endothelial dysfunction; Vascular tone; BMP signaling
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This article provides a comprehensive review of the implication of the BMP-9/BMPR-II/ALK1/endoglin pathway in the pathophysiology of vascular diseases such as pulmonary arterial hypertension, hereditary hemorrhagic telangiectasia, and hepatopulmonary syndrome.
The signaling pathway of the bone morphogenetic protein (BMP)-9 binding to the endothelial receptor BMP receptor type II (BMPR-II), activin receptor-like kinase-1 (ALK1) and the coreceptor endoglin is essential to maintain the pulmonary vascular integrity. Dysregula-tion of this pathway is implicated in numerous vascular diseases, such as pulmonary arterial hypertension (PAH), hereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syn-drome (HPS). This article aims to provide a comprehensive review of the implication of the BMP-9/BMPR-II/ALK1/endoglin pathway in the pathophysiology of these diseases. (c) 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.
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