Lindholm D, Wootz H, Korhonen L
Department of Neuroscience, Unit of Neurobiology, Uppsala University, Biomedical Centre, Box 587, S-751 23 Uppsala, Sweden.
Cell Death Differ. 2006 Mar;13(3):385-92. doi: 10.1038/sj.cdd.4401778.
Endoplasmic reticulum (ER) stress is caused by disturbances in the structure and function of the ER with the accumulation of misfolded proteins and alterations in the calcium homeostasis. The ER response is characterized by changes in specific proteins, causing translational attenuation, induction of ER chaperones and degradation of misfolded proteins. In case of prolonged or aggravated ER stress, cellular signals leading to cell death are activated. ER stress has been suggested to be involved in some human neuronal diseases, such as Parkinson's disease, Alzheimer's and prion disease, as well as other disorders. The exact contributions to and casual effects of ER stress in the various disease processes, however, are not known. Here we will discuss the possible role of ER stress in neurodegenerative diseases, and highlight current knowledge in this field that may reveal novel insight into disease mechanisms and help to design better therapies for these disorders.
内质网(ER)应激是由内质网结构和功能紊乱、错误折叠蛋白积累以及钙稳态改变引起的。内质网反应的特征是特定蛋白质发生变化,导致翻译衰减、内质网伴侣蛋白的诱导以及错误折叠蛋白的降解。在长时间或严重内质网应激的情况下,会激活导致细胞死亡的细胞信号。内质网应激已被认为与一些人类神经疾病有关,如帕金森病、阿尔茨海默病和朊病毒病,以及其他疾病。然而,内质网应激在各种疾病过程中的确切作用和因果效应尚不清楚。在这里,我们将讨论内质网应激在神经退行性疾病中的可能作用,并强调该领域的当前知识,这些知识可能揭示疾病机制的新见解,并有助于设计针对这些疾病的更好疗法。