Dysfonction de la signalisation endoth?liale du BMP-9 dans les maladies vasculaires pulmonaires

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.

Automated summary

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.