肺动脉高压相关因子研究进展

Research progress of factors related with pulmonary arterial hypertension

肺动脉高压以肺血管阻力升高为特征，由于小肺动脉血管平滑肌细胞增殖，肺动脉血管阻力升高，导致右心室衰竭直至死亡。肺动脉高压的发生不能以单一的病理生理理论来解释，而是涉及多种分子介质、遗传因素和平滑肌细胞、内皮细胞等多种细胞异常。最近的研究表明多种因子通过不同的途径参与了肺动脉高压的发病机制，包括骨形成蛋白Ⅱ型受体、Notch3、过氧化物酶增殖体激活受体γ、PTEN等。

Pulmonary arterial hypertension （PAH） is characterized by excessive vascular resistance. Smooth muscle cell proliferation in small pulmonary arteries, leading to elevation of pulmonary vascular resistance, causes right ventricular failure and death. Multiple pathogenic pathways have been implicated in the development of PAH, including those at the molecular and genetic levels and in the smooth muscle and endothelial cells. Recent advances indicate that molecular factors such as Notch3, bone morphogenetie protein typeⅡreceptor, peroxisome proliferator-activated receptor gamma and PTEN can have a favorable impact on a variety of pathways involved in the pathogenesis of PAH.