Endoplasmic reticulum stress in disorders of myelinating cells.
Endoplasmic reticulum stress in disorders of myelinating cells.
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DOI：
10.1038/nn.2273
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发表时间：
2009-04
影响因子：
25
通讯作者：
Popko, Brian
中科院分区：
文献类型：
--
作者：
Lin, Wensheng;Popko, Brian
髓鞘细胞，中枢神经系统的少突胶质细胞和PNS的雪旺细胞，在髓鞘形成过程中产生大量的质膜，使它们特别容易受到分泌途径的破坏。内质网应激是由未折叠或错误折叠蛋白的积累引发的，激活未折叠蛋白反应（UPR），使细胞适应应激。如果这种适应性反应不足，UPR会激活凋亡程序来消除受影响的细胞。最近的观察表明，髓鞘细胞内质网应激在各种髓鞘疾病的发病机制中起重要作用，包括charco - marie - tooth病（CMT）、Pelizaeus-Merzbacher病（PMD）、消失白质病（VWMD），以及最常见的髓鞘疾病多发性硬化症（MS）。更好地了解髓鞘细胞内质网应激为设计新的治疗策略以促进这些疾病中的髓鞘细胞存活奠定了基础。
Myelinating cells, oligodendrocytes in the CNS and Schwann cells in the PNS, produce an enormous amount of plasma membrane during the myelination process, making them particularly susceptible to disruptions to the secretory pathway. ER stress, initiated by the accumulation of unfolded or misfolded proteins, activates the unfolded protein response (UPR), which adapts cells to the stress. If this adaptive response is insufficient, the UPR activates an apoptotic program to eliminate the affected cells. Recent observations suggest that ER stress in myelinating cells plays an important role in the pathogenesis of various disorders of myelin, including Charcot-Marie-Tooth disease (CMT), Pelizaeus-Merzbacher disease (PMD), Vanishing White Matter Disease (VWMD), as well as in the most common disorder of myelin, multiples sclerosis (MS). A better understanding of ER stress in myelinating cells has laid the groundwork for the design of novel therapeutic strategies to promote myelinating cell survival in these disorders.
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影响因子：
3.7
作者：
Kantor L;Pinchasi D;Mintz M;Hathout Y;Vanderver A;Elroy-Stein O
通讯作者：
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影响因子：
16
作者：
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影响因子：
64.8
作者：
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通讯作者：
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影响因子：
3.2
作者：
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通讯作者：
Lassmann, H
DOI：
10.1073/pnas.152621799
发表时间：
2002-07-23
影响因子：
11.1
作者：
Dickson, KM;Bergeron, JJM;Snipes, GJ
通讯作者：
Snipes, GJ