中国神经再生研究(英文版) ›› 2025, Vol. 20 ›› Issue (1): 6-20.doi: 10.4103/NRR.NRR-D-23-00995

• 综述:退行性病与再生 • 上一篇    下一篇

内质网自噬对神经退行性疾病的调节与功能

  

  • 出版日期:2025-01-15 发布日期:2025-01-15
  • 基金资助:
    国家自然科学基金项目(92049120和81870897);STI2030重大项目(2021ZD0204001);广东省孤独症诊疗新工具开发重点项目(2018B030335001);江苏省自然科学基金项目(BK20181436);国家重大新药开发重大科技专项(2019ZX09301102);苏州大学第二附属医院学科建设项目(XKTJ-TD202003);中德合作流动计划项目(M-0679);苏州市科技计划项目(SKY2022161);苏州大学医学中心附属第二医院神经系统疾病研究项目(ND2023A01)

Regulation and function of endoplasmic reticulum autophagy in neurodegenerative diseases

Xiu-Yun Zhao1, 2, De-En Xu3, Ming-Lei Wu1, 2, Ji-Chuan Liu1, 2, Zi-Ling Shi1, 2, Quan-Hong Ma1, 2, *   

  1. 1Department of Neurology and Clinical Research Center of Neurological Disease, the Second Affiliated Hospital of Soochow University, Suzhou, Jiangsu Province, China; 2Institute of Neuroscience & Jiangsu Key Laboratory of Translational Research and Therapy for Neuro-Psycho-Diseases, Soochow University, Suzhou, Jiangsu Province, China; 3Department of Neurology, Jiangnan University Medical Center, Wuxi, Jiangsu Province, China
  • Online:2025-01-15 Published:2025-01-15
  • Contact: Quan-Hong Ma, PhD, maquanhong@suda.edu.cn.
  • Supported by:
    This work was supported by the National Natural Science Foundation of China, Nos. 92049120 and 81870897; STI2030-Major Projects, No. 2021ZD0204001; Guangdong Key Project for Development of New Tools for the Diagnosis and Treatment of Autism, No. 2018B030335001; the Natural Science Foundation of Jiangsu Province, No. BK20181436; the National Major Scientific and Technological Special Project for Significant New Drug Development, No. 2019ZX09301102; the Discipline Construction Program of the Second Affiliated Hospital of Soochow University, No. XKTJ-TD202003; Sino-German Cooperation Mobility Programme, No. M-0679; the Science and Technology Project of Suzhou, No. SKY2022161; and Research Project of Neurological Diseases of the Second Affiliated Hospital of Soochow University Medical Center, No. ND2023A01 (all to QHM).

摘要:

内质网作为细胞中至关重要的细胞器,调节着细胞内多种生命活动。内质网自噬作为内质网质量控制系统之一,可通过控制内质网转运、内质网重塑和内质网蛋白稳定,在维持内质网稳态方面中发挥重要的作用。文章描述了内质网质量控制系统,并重点关注了内质网自噬在响应细胞需求时的空间和时间上的分子调控机制,还总结了内质网自噬缺陷或异常参与神经退行性疾病发病机制的证据。

https://orcid.org/0000-0001-9392-8956 (Quan-Hong Ma)

关键词: 自噬, 内质网, 内质网相关降解, 内质网自噬受体, 内质网自噬, 内质网质量控制系统, 神经退行性变, 神经退行性疾病, 选择性自噬, 未折叠蛋白反应

Abstract: The endoplasmic reticulum, a key cellular organelle, regulates a wide variety of cellular activities. Endoplasmic reticulum autophagy, one of the quality control systems of the endoplasmic reticulum, plays a pivotal role in maintaining endoplasmic reticulum homeostasis by controlling endoplasmic reticulum turnover, remodeling, and proteostasis. In this review, we briefly describe the endoplasmic reticulum quality control system, and subsequently focus on the role of endoplasmic reticulum autophagy, emphasizing the spatial and temporal mechanisms underlying the regulation of endoplasmic reticulum autophagy according to cellular requirements. We also summarize the evidence relating to how defective or abnormal endoplasmic reticulum autophagy contributes to the pathogenesis of neurodegenerative diseases. In summary, this review highlights the mechanisms associated with the regulation of endoplasmic reticulum autophagy and how they influence the pathophysiology of degenerative nerve disorders. This review would help researchers to understand the roles and regulatory mechanisms of endoplasmic reticulum-phagy in neurodegenerative disorders.

Key words: autophagy, endoplasmic reticulum, endoplasmic reticulum autophagy, endoplasmic reticulum quality control system, endoplasmic reticulum receptors, endoplasmic reticulum-associated degradation, neurodegeneration, neurodegenerative disease, selective autophagy, unfolded protein response