Department of Biochemistry, University of Utah School of Medicine, Salt Lake City, UT 84112, USA.
Int J Biochem Cell Biol. 2011 Oct;43(10):1422-6. doi: 10.1016/j.biocel.2011.06.002. Epub 2011 Jun 12.
Progressive mitochondrial failure is tightly associated with the onset of many age-related human pathologies. This tight connection results from the double-edged sword of mitochondrial respiration, which is responsible for generating both ATP and ROS, as well as from risks that are inherent to mitochondrial biogenesis. To prevent and treat these diseases, a precise understanding of the mechanisms that maintain functional mitochondria is necessary. Mitochondrial protein quality control is one of the mechanisms that protect mitochondrial integrity, and increasing evidence implicates the cytosolic ubiquitin/proteasome system (UPS) as part of this surveillance network. In this review, we will discuss our current understanding of UPS-dependent mitochondrial protein degradation, its roles in diseases progression, and insights into future studies.
进行性线粒体衰竭与许多与年龄相关的人类疾病的发生密切相关。这种紧密联系源于线粒体呼吸的双刃剑效应，线粒体呼吸既负责产生 ATP 和 ROS，又带来了与线粒体生物发生相关的固有风险。为了预防和治疗这些疾病，有必要精确了解维持功能线粒体的机制。线粒体蛋白质量控制是保护线粒体完整性的机制之一，越来越多的证据表明细胞质泛素/蛋白酶体系统（UPS）是这个监控网络的一部分。在这篇综述中，我们将讨论我们对 UPS 依赖性线粒体蛋白降解的现有理解，以及它在疾病进展中的作用和对未来研究的启示。
