线粒体是生命必需的亚细胞细胞器。除了产生能量的作用之外，线粒体还控制着各种生理机制，包括能量产生、代谢过程、细胞凋亡事件和免疫反应。线粒体还含有易受各种形式损伤的遗传物质。线粒体双链断裂（DSB）是细胞核迅速修复的毒性损伤。然而，哺乳动物线粒体中 DSB 修复的意义仍存在争议。这篇综述提出了现有研究的最新观点，即线粒体 DNA DSB 从分子到细胞水平的影响。我们讨论了线粒体 DNA 损伤在衰老、综合应激反应和先天免疫等调节过程中的关键功能。最后，我们讨论了线粒体 DNA DSB 在介导电离辐射的细胞后果中的潜在作用，电离辐射是治疗实体瘤的护理标准。© 作者 2024。由牛津大学出版社出版。版权所有。如需权限，请发送电子邮件至：journals.permissions@oup.com。

Mitochondria are subcellular organelles essential for life. Beyond their role in producing energy, mitochondria govern various physiological mechanisms, encompassing energy generation, metabolic processes, apoptotic events, and immune responses. Mitochondria also contain genetic material that is susceptible to various forms of damage. Mitochondrial double-stranded breaks (DSB) are toxic lesions that the nucleus repairs promptly. Nevertheless, the significance of DSB repair in mammalian mitochondria is controversial. This review presents an updated view of the available research on the consequences of mitochondrial DNA DSB from the molecular to the cellular level. We discuss the crucial function of mitochondrial DNA damage in regulating processes such as senescence, integrated stress response, and innate immunity. Lastly, we discuss the potential role of mitochondrial DNA DSB in mediating the cellular consequences of ionizing radiations, the standard of care in treating solid tumors.© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.