Zhang Junming, Yan Huimin, Wang Yan, Yue Xian, Wang Meng, Liu Limin, Qiao Pengfei, Zhu Yixuan, Li Zhichao
Faculty of Life Science & Medicine, Northwest University, Xi'an, 710127, Shaanxi, China.
Mol Cell Biochem. 2025 Mar;480(3):1407-1429. doi: 10.1007/s11010-024-05096-9. Epub 2024 Sep 10.
Pulmonary hypertension (PH) is heterogeneous diseases that can lead to death due to progressive right heart failure. Emerging evidence suggests that, in addition to its role in ATP production, changes in mitochondrial play a central role in their pathogenesis, regulating integrated metabolic and signal transduction pathways. This review focuses on the basic principles of mitochondrial redox status in pulmonary vascular and right ventricular disorders, a series of dysfunctional processes including mitochondrial quality control (mitochondrial biogenesis, mitophagy, mitochondrial dynamics, mitochondrial unfolded protein response) and mitochondrial redox homeostasis. In addition, we will summarize how mitochondrial renewal and dynamic changes provide innovative insights for studying and evaluating PH. This will provide us with a clearer understanding of the initial signal transmission of mitochondria in PH, which would further improve our understanding of the pathogenesis of PH.
肺动脉高压（PH）是一类异质性疾病，可因进行性右心衰竭导致死亡。新出现的证据表明，线粒体除了在三磷酸腺苷（ATP）生成中发挥作用外，其变化在肺动脉高压的发病机制中也起着核心作用，调节着整合的代谢和信号转导通路。本综述聚焦于肺血管和右心室疾病中线粒体氧化还原状态的基本原理，一系列功能失调过程，包括线粒体质量控制（线粒体生物发生、线粒体自噬、线粒体动力学、线粒体未折叠蛋白反应）和线粒体氧化还原稳态。此外，我们将总结线粒体更新和动态变化如何为研究和评估肺动脉高压提供创新性见解。这将使我们更清楚地了解肺动脉高压中线粒体的初始信号传递，从而进一步增进我们对肺动脉高压发病机制的理解。
