中国神经再生研究(英文版) ›› 2022, Vol. 17 ›› Issue (12): 2563-2575.doi: 10.4103/1673-5374.339473

• 综述:神经损伤修复保护与再生 • 上一篇    下一篇

线粒体应激反应:“化身博士”的神经元疾病

  

  • 出版日期:2022-12-15 发布日期:2022-05-05

The “mitochondrial stress responses”: the “Dr. Jekyll and Mr. Hyde” of neuronal disorders

Simone Patergnani1, Giampaolo Morciano1, Marianna Carinci1, Sara Leo1, Paolo Pinton1, 2, Alessandro Rimessi1, 2, *   

  1. 1Department of Medical Sciences, Section of Experimental Medicine, Laboratory for Technologies of Advanced Therapies, University of Ferrara, Ferrara, Italy; 2Center of Research for Innovative Therapies in Cystic Fibrosis, University of Ferrara, Ferrara, Italy
  • Online:2022-12-15 Published:2022-05-05
  • Contact: Alessandro Rimessi, PhD, alessandro.rimessi@unife.it.
  • Supported by:
    This work was supported by grants from local funds from University of Ferrara, FIR-2021, the Italian Ministry of Health (GR-2016-02364602) and the Italian Ministry of Education, University and Research (PRIN Grant 2017XA5J5N) (to AR); grants from Italian Association for Cancer Research (AIRC, IG-23670), Telethon (GGP11139B), local funds from the University of Ferrara, and the Italian Ministry of Education, University and Research (PRIN Grant 2017E5L5P3) (to PP): Associazione Ricerca Oncologica Sperimentale Estense (A-ROSE).

摘要: https://orcid.org/0000-0001-7091-5765 (Alessandro Rimessi)

Abstract: Neuronal disorders are associated with a profound loss of mitochondrial functions caused by various stress conditions, such as oxidative and metabolic stress, protein folding or import defects, and mitochondrial DNA alteration. Cells engage in different coordinated responses to safeguard mitochondrial homeostasis. In this review, we will explore the contribution of mitochondrial stress responses that are activated by the organelle to perceive these dangerous conditions, keep them under control and rescue the physiological condition of nervous cells. In the sections to come, particular attention will be dedicated to analyzing how compensatory mitochondrial hyperfusion, mitophagy, mitochondrial unfolding protein response, and apoptosis impact human neuronal diseases. Finally, we will discuss the relevance of the new concept: the “mito-inflammation”, a mitochondria-mediated inflammatory response that is recently found to cover a relevant role in the pathogenesis of diverse inflammatory-related diseases, including neuronal disorders.

Key words: Alzheimer’s disease, apoptosis, mitochondrial dynamics, mito-inflammation, mitophagy, multiple sclerosis, neurodegeneration, Parkinson’s disease, UPRmt