Pulmonary arterial hypertension (PAH) is a disease with complex pathobiology, significant morbidity and mortality, and remains without a cure. It is characterized by vascular remodelling associated with uncontrolled proliferation of pulmonary artery smooth muscle cells, endothelial cell proliferation and dysfunction, and endothelial-to-mesenchymal transition, leading to narrowing of the vascular lumen, increased vascular resistance and pulmonary arterial pressure, which inevitably results in right heart failure and death. There are multiple molecules and signalling pathways that are involved in the vascular remodelling, including non-coding RNAs, i.e. microRNAs and long non-coding RNAs (lncRNAs). It is only in recent years that the role of lncRNAs in the pathobiology of pulmonary vascular remodelling and right ventricular dysfunction is being vigorously investigated. In this review, we have summarized the current state of knowledge about the role of lncRNAs as key drivers and gatekeepers in regulating major cellular and molecular trafficking involved in the pathogenesis of PAH. In addition, we have discussed the limitations and challenges in translating lncRNA research in vivo and in therapeutic applications of lncRNAs in PAH.

中文翻译：

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肺动脉高压的病理生物学：长链非编码 RNA 的作用。

肺动脉高压 (PAH) 是一种病理学复杂、发病率和死亡率显着的疾病，并且仍然无法治愈。其特点是血管重构与肺动脉平滑肌细胞不受控制的增殖、内皮细胞增殖和功能障碍以及内皮向间充质转化相关，导致血管腔变窄、血管阻力和肺动脉压升高，不可避免地导致在右心衰竭和死亡。血管重塑涉及多种分子和信号通路，包括非编码RNAs，即microRNAs和长链非编码RNAs(lncRNAs)。只是在最近几年，lncRNAs 在肺血管重构和右心室功能障碍的病理生物学中的作用才被大力研究。在这篇综述中，我们总结了关于 lncRNA 在调节参与 PAH 发病机制的主要细胞和分子运输中作为关键驱动因素和看门人的作用的知识现状。此外，我们还讨论了翻译 lncRNA 研究的局限性和挑战lncRNAs 在 PAH中的体内和治疗应用。