1] Biomedical Neuroscience Institute, Faculty of Medicine, University of Chile, Santiago, Chile. [2] Institute of Biomedical Sciences, Center for Molecular Studies of the Cell, Program of Cellular and Molecular Biology, University of Chile, Santiago, Chile. [3] Neurounion Biomedical Foundation, Santiago, Chile. [4] Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, Massachusetts 02115, USA.
Laboratory of Molecular Biology of the Cell, CNRS UMR5239, Ecole Normale Supérieure de Lyon, UMS3444 Biosciences Lyon Gerland, University of Lyon, Lyon 69364, France.
Nat Rev Neurosci. 2014 Apr;15(4):233-49. doi: 10.1038/nrn3689. Epub 2014 Mar 12.
The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well characterized in non-neuronal cells, a proliferation of papers over the past few years has revealed a key role for the UPR in normal neuronal function and as an important driver of neurodegenerative diseases. A complex scenario is emerging in which distinct UPR signalling modules have specific and even opposite effects on neurodegeneration depending on the disease context. Here, we provide an overview of the most recent findings addressing the biological relevance of ER stress in the nervous system.
未折叠蛋白反应（UPR）是一种细胞内稳态机制，通过该机制细胞可调节内质网（ER）中错误折叠蛋白的水平。尽管在非神经元细胞中该机制已经得到了很好的描述，但近年来大量文献揭示了 UPR 在正常神经元功能中的关键作用，以及其作为神经退行性疾病重要驱动因素的作用。目前，一种复杂的情况正在出现，即不同的 UPR 信号模块根据疾病背景对神经退行性变具有特定的甚至相反的影响。在这里，我们提供了一个概述，介绍了最新的关于 ER 应激在神经系统中的生物学相关性的研究结果。
