Department of Physiology, College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America.
Mol Med. 2014 Apr 22;20(1):191-201. doi: 10.2119/molmed.2013.00165.
Pulmonary arterial hypertension (PAH) is characterized by a persistent elevation of pulmonary arterial pressure and pulmonary arterial remodeling with unknown etiology. Current therapeutics available for PAH are primarily directed at reducing the pulmonary blood pressure through their effects on the endothelium. It is well accepted that pulmonary arterial remodeling is primarily due to excessive pulmonary arterial smooth muscle cell (PASMC) proliferation that leads to narrowing or occlusion of the pulmonary vessels. Future effective therapeutics will be successful in reversing the vascular remodeling in the pulmonary arteries and arterioles. The purpose of this review is to provide updated information on molecular mechanisms involved in pulmonary arterial remodeling with a focus on growth factors, transcription factors, and epigenetic pathways in PASMC proliferation. In addition, this review will highlight novel therapeutic strategies for potentially reversing PASMC proliferation.
肺动脉高压（PAH）的特征是肺动脉压力持续升高和肺动脉重构，其病因不明。目前可用于治疗 PAH 的方法主要通过作用于内皮细胞来降低肺动脉压。人们普遍认为，肺动脉重构主要是由于肺动脉平滑肌细胞（PASMC）过度增殖，导致肺血管狭窄或闭塞。未来有效的治疗方法将成功逆转肺动脉和小动脉的血管重构。本综述的目的是提供有关肺动脉重构分子机制的最新信息，重点介绍生长因子、转录因子和 PASMC 增殖中的表观遗传途径。此外，本综述还将重点介绍潜在逆转 PASMC 增殖的新治疗策略。
