'Unfolding' pathways in neurodegenerative disease
'Unfolding' pathways in neurodegenerative disease
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DOI：
10.1016/s0166-2236(03)00197-8
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发表时间：
2003-08-01
影响因子：
15.9
通讯作者：
Trojanowski, JQ
中科院分区：
文献类型：
--
作者：
Forman, MS;Lee, VMY;Trojanowski, JQ
内质网通过启动一系列被称为“未折叠蛋白反应”（UPR）的事件来应对压力。白质营养不良Pelizaeus-Merzbacher病中错误折叠蛋白的积累激活了这种应激反应，导致少突胶质细胞凋亡。虽然UPR是否在典型的神经退行性疾病如阿尔茨海默病中起机制作用仍不确定，但这是有可能的，因为错误折叠的蛋白质与这些疾病的发病机制直接相关。
The endoplasmic reticulum responds to stress by initiating a cascade of events known as the 'unfolded-protein response' (UPR). The accumulation of misfolded proteins in the leukodystrophy Pelizaeus-Merzbacher disease activates this stress response, resulting in apoptosis of oligodendrocytes. Although it remains uncertain whether the UPR plays a mechanistic role in prototypical neurodegenerative disorders such as Alzheimer's disease, this is plausible because misfolded proteins are directly implicated in the pathogenesis of these disorders.