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作者

Zhihong Xin, Junfu Wang, Susu Li, Chao Sun, Wan Jiang, Qian Xin, Jue Wang, Tonggnag Qi, Kailin Li, Zhaohua Zhang, Yun Luan

作者单位

Clinical laboratory, The Second Hospital, Cheeloo College of Medicine, Shandong University. College of pharmacy, Shandong First Medical University & Shandong Academy of Medical Sciences, Taian, Shandong, 271000, China. Department of Pediatrics, The Second Hospital, Cheeloo College of Medicine, Shandong University. Institute of Medical Science, The Second Hospital, Cheeloo College of Medicine, Shandong University, No. 247, Beiyuan Dajie, Jinan, 250033, P.R. China. Affiliations

刊名

Clinical and experimental hypertension (New York, N.Y. : 1993)

年份

2022

卷号

Vol.44 No.2

页码

175-180

ISSN

1525-6006

关键词

BMP PAH PASMCs Wnt5A right ventricular failure.

摘要

Pulmonary arterial hypertension is a chronic disease characterized by a progressive elevation in mean pulmonary arterial pressure. This occurs due to abnormal remodeling of small peripheral lung vasculature resulting in progressive occlusion of the artery lumen that eventually causes right heart failure and death. Current therapeutic options for PAH are limited and focused mainly on reversal of pulmonary vasoconstriction and proliferation of vascular cells. Although these treatments can relieve...更多

Pulmonary arterial hypertension is a chronic disease characterized by a progressive elevation in mean pulmonary arterial pressure. This occurs due to abnormal remodeling of small peripheral lung vasculature resulting in progressive occlusion of the artery lumen that eventually causes right heart failure and death. Current therapeutic options for PAH are limited and focused mainly on reversal of pulmonary vasoconstriction and proliferation of vascular cells. Although these treatments can relieve disease symptoms, PAH remains a progressive lethal disease.Bone morphogenetic proteins and their receptors were required for PAH-induced right ventricular hypertrophy. Emerging data suggest that restoration of BMP type II receptor signaling in PAH is a promising alternative that could prevent and reverse pulmonary vascular remodeling. BMPR2 mutations have been identified in >70% of familial and roughly 15% of sporadic PAH cases. Wingless are a family of secreted glycoproteins with varying expression patterns and a range of functions, Wnt signaling pathway is divided into canonical signaling pathway and non-canonical signaling pathway. A recent study reports that interaction between BMP and Wnt closely associated with lung development, those cascade coordination regulation stem cell fate which determine lung branching morphogenes. The promoting effect of BMPR2 on proliferation, survival, and motility of endothelial cells was through recruiting Wnts signaling pathway, the interaction between BMP and Wnt closely associated with lung development.Therefore, in this review, we outline the latest advances of BMP and Wnt signaling pathway in the pathogenesis of PAH and disease progression.收起

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