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肺动脉高压（PH）是一种以肺血管压力进行性升高为主要特征，并最终导致右心功能障碍甚至衰竭的致命性疾病。其核心病理改变为肺小动脉的异常收缩和血管重构。近年来，内质网应激（ERS）作为细胞应对内外环境压力下产生的关键适应性反应，在PH的发生与发展中发挥关键作用。ERS通过激活肌醇需求酶1α(IRE1α)、RNA样ER激酶（PERK）和活化转录因子6(ATF6)等主要信号通路，直接参与调控PH中细胞的生物学行为异常改变和血管重构等病理过程。本文拟系统探讨ERS在PH发病机制中的具体作用，并分析其作为潜在治疗策略的可行性，以期为PH的临床干预提供新的理论依据。
Abstract：Pulmonary hypertension(PH) is a life-threatening condition characterized by a progressive elevation in pulmonary vascular pressure, ultimately leading to right ventricular dysfunction and failure. The core pathological features of PH include abnormal contraction of small pulmonary arteries and vascular remodeling. In recent years, endoplasmic reticulum stress(ERS), a critical adaptive response of cells to internal and external stressors, has been shown to play a key role in the development and progression of PH. Through the activation of major signaling pathways such as protein kinase R-like endoplasmic reticulum kinase(PERK), inositol-requiring enzyme 1α(IRE1α), and activating transcription factor 6(ATF6), ERS is directly involved in regulating aberrant cellular behaviors and vascular remodeling in PH. This review systematically discusses the specific role of ERS in the pathogenesis of PH and evaluates its potential as a therapeutic target, aiming to provide a theoretical foundation for novel clinical interventions in PH.
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基本信息:
DOI：10.19405/j.cnki.issn1000-1492.2026.08.023
中图分类号:R544.1
引用信息:
[1]曹煜熙,薛志峰,周思仪,等.内质网应激与肺动脉高压：从病理机制到靶向干预的新视角[J].安徽医科大学学报,2026(08):1498-1504.DOI:10.19405/j.cnki.issn1000-1492.2026.08.023.
基金信息:
国家自然科学基金项目(编号:82570084、82405380); 山西省医学重点科研项目(编号:2023XM003); 山西省自然科学基金项目(编号:202203021221238); 山西省中医药管理局科研课题(编号:2023ZYYB027)
2026-07-23
2026-07-23
2026-07-23