Bone morphogenetic protein receptor Ⅱ signaling pathway and pulmonary arterial hypertension:updates and expectations / 中国药理学与毒理学杂志

ABSTRACT

The identification of the relationships between mutations of the bone morphogenetic protein type Ⅱ receptor (BMPR2) and pulmonary arterial hypertension (PAH) has been considered to be one of the most significant discoveries in this area in the 21st century. And BMPR2 mutation is responsible for the majority of hereditary PAH as well as some of idiopathic PAH. Furthermore, clinical and animal expreimental research over the past few years has revealed that BMPR2 signaling pathway plays a critical role in the initiation and progress of PAH, by participateing in the pathogenesis of PAH. In addition, the potential that BMPR2 signaling pathway is used as a therapeutic target is being evaluated. This review summarizes our current understanding of the role of BMPR2 mutations in PAH from the perspectives of genetics, epigenetics, inflammation as well as interactions with other significant pathways.