[Dysfunction of endothelial BMP-9 signaling in pulmonary vascular disease]

Abstract

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. Dysregulation of this pathway is implicated in numerous vascular diseases, such as pulmonary arterial hypertension (PAH), hereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syndrome (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.

Keywords: BMP signaling; Endothelial dysfunction; Pulmonary vascular remodeling; Remodelage vasculaire pulmonaire, Dysfonction endothéliale, Tonus vasculaire, Voie de signalisation des BMP; Vascular tone.