Mitochondrial Unfolded Protein Response and Integrated Stress Response as Promising Therapeutic Targets for Mitochondrial Diseases

作者：Hedong Lu, Xiaolei Wang, Min Li, Dongmei Ji, Dan Liang, Chunmei Liang, Yajing Liu, Zhiguo Zhang, Yunxia Cao, Weiwei Zou · 发表于：Cells · 年份：2022 · DOI：10.3390/cells12010020 · 被引用次数：42 · 研究领域：Mitochondrial Function and Pathology、Endoplasmic Reticulum Stress and Disease、ATP Synthase and ATPases Research

The development and application of high-throughput omics technologies have enabled a more in-depth understanding of mitochondrial biosynthesis metabolism and the pathogenesis of mitochondrial diseases. In accordance with this, a host of new treatments for mitochondrial disease are emerging. As an essential pathway in maintaining mitochondrial proteostasis, the mitochondrial unfolded protein response (UPRmt) is not only of considerable significance for mitochondrial substance metabolism but also plays a fundamental role in the development of mitochondrial diseases. Furthermore, in mammals, the integrated stress response (ISR) and UPRmt are strongly coupled, functioning together to maintain mitochondrial function. Therefore, ISR and UPRmt show great application prospects in the treatment of mitochondrial diseases. In this review, we provide an overview of the molecular mechanisms of ISR and UPRmt and focus on them as potential targets for mitochondrial disease therapy.