The unfolded protein response in multiple sclerosis.
The unfolded protein response in multiple sclerosis.
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DOI：
10.3389/fnins.2015.00264
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发表时间：
2015
影响因子：
4.3
通讯作者：
Lin W
中科院分区：
文献类型：
--
作者：
Stone S;Lin W
未折叠蛋白反应（UPR）是对内质网（ER）应激的反应，内质网应激是由未折叠或错误折叠蛋白在ER中的积累引起的。UPR由促进细胞保护功能以纠正ER应激的三个信号通路组成;然而，如果ER应激不能被解决，则UPR导致受影响细胞的凋亡。UPR是各种人类疾病的重要特征，包括多发性硬化症（MS）。最近的研究表明，UPR的几种组分在MS病变的多种细胞类型中上调，包括少突胶质细胞、T细胞、小胶质细胞/巨噬细胞和星形胶质细胞。从动物模型的研究，特别是实验性自身免疫性脑脊髓炎（EAE）和cuprizone模型的研究数据，意味着一个重要的作用的UPR激活少突胶质细胞在MS的发展。在这篇综述中，我们将涵盖目前的文献上的UPR和证据，其在MS的发展中的作用。
The unfolded protein response (UPR) occurs in response to endoplasmic reticulum (ER) stress caused by the accumulation of unfolded or misfolded proteins in the ER. The UPR is comprised of three signaling pathways that promote cytoprotective functions to correct ER stress; however, if ER stress cannot be resolved the UPR results in apoptosis of affected cells. The UPR is an important feature of various human diseases, including multiple sclerosis (MS). Recent studies have shown several components of the UPR are upregulated in the multiple cell types in MS lesions, including oligodendrocytes, T cells, microglia/macrophages, and astrocytes. Data from animal model studies, particularly studies of experimental autoimmune encephalomyelitis (EAE) and the cuprizone model, imply an important role of the UPR activation in oligodendrocytes in the development of MS. In this review we will cover current literature on the UPR and the evidence for its role in the development of MS.