Molecular Biology and Biochemistry, Gottfried Schatz Research Center, Medical University of Graz, Neue Stiftingtalstraße 6/6, 8010 Graz, Austria.
Division of Biological Sciences and Center for Structural and Functional Neuroscience, The University of Montana, 32 Campus Drive, HS410, Missoula, MT 59812-4824, U.S.A.
Biol Rev Camb Philos Soc. 2019 Apr;94(2):610-628. doi: 10.1111/brv.12469. Epub 2018 Oct 19.
The endoplasmic reticulum (ER) is a functionally and morphologically complex cellular organelle largely responsible for a variety of crucial functions, including protein folding, maturation and degradation. Furthermore, the ER plays an essential role in lipid biosynthesis, dynamic Ca storage, and detoxification. Malfunctions in ER-related processes are responsible for the genesis and progression of many diseases, such as heart failure, cancer, neurodegeneration and metabolic disorders. To fulfill many of its vital functions, the ER relies on a sufficient energy supply in the form of adenosine-5'-triphosphate (ATP), the main cellular energy source. Despite landmark discoveries and clarification of the functional principles of ER-resident proteins and key ER-related processes, the mechanism underlying ER ATP transport remains somewhat enigmatic. Here we summarize ER-related ATP-consuming processes and outline our knowledge about the nature and function of the ER energy supply.
内质网（ER）是一种在功能和形态上都非常复杂的细胞器，主要负责多种关键功能，包括蛋白质折叠、成熟和降解。此外，内质网在脂质生物合成、动态钙储存和解毒中也发挥着重要作用。与内质网相关过程的功能障碍是许多疾病（如心力衰竭、癌症、神经退行性疾病和代谢紊乱）发生和发展的原因。为了完成许多重要功能，内质网依赖于以三磷酸腺苷（ATP）形式提供的充足能量供应，ATP 是细胞的主要能量来源。尽管对内质网驻留蛋白和关键内质网相关过程的功能原理进行了里程碑式的发现和澄清，但内质网 ATP 转运的机制仍然有些神秘。在这里，我们总结了与内质网相关的消耗 ATP 的过程，并概述了我们对内质网能量供应性质和功能的了解。
