Eukaryotic cells must accurately monitor the integrity of the mitochondrial network to overcome environmental insults and respond to physiological cues. The mitochondrial unfolded protein response (UPRmt) is a mitochondrial-to-nuclear signaling pathway that maintains mitochondrial proteostasis, mediates signaling between tissues, and regulates organismal aging. Aberrant UPRmt signaling is associated with a wide spectrum of disorders, including congenital diseases as well as cancers and neurodegenerative diseases. Here, we review recent research into the mechanisms underlying UPRmt signaling in Caenorhabditis elegans and discuss emerging connections between the UPRmt signaling and a translational regulation program called the 'integrated stress response'. Further study of the UPRmt will potentially enable development of new therapeutic strategies for inherited metabolic disorders and diseases of aging.

中文翻译：

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将线粒体 UPR 纳入综合应激反应中。

真核细胞必须准确监测线粒体网络的完整性，以克服环境损害并对生理信号做出反应。线粒体未折叠蛋白反应 (UPRmt) 是一种线粒体到核的信号传导途径，可维持线粒体蛋白质稳态、介导组织之间的信号传导并调节机体衰老。异常的 UPRmt 信号传导与多种疾病有关，包括先天性疾病以及癌症和神经退行性疾病。在这里，我们回顾了最近对秀丽隐杆线虫 UPRmt 信号传导机制的研究，并讨论了 UPRmt 信号传导与称为“综合应激反应”的翻译调节程序之间的新联系。对 UPRmt 的进一步研究将有可能为遗传性代谢紊乱和衰老疾病开发新的治疗策略。