Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
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DOI：
10.1093/abbs/gmt014
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发表时间：
2013-06
影响因子：
3.7
通讯作者：
Zhiqi Song;Deming Zhao;Lifeng Yang
中科院分区：
文献类型：
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作者：
Zhiqi Song;Deming Zhao;Lifeng Yang
传染性海绵状脑病是指一组传染性神经退行性疾病，具有全新的传播机制和病理生理学，包括突触损伤、树突萎缩、空泡化和小胶质细胞活化。广泛的神经元丢失是朊病毒病的慢性脑退化和致命结果的主要原因。作为病理改变的最终结果，神经元死亡是所有朊病毒疾病的显著特征。朊病毒疾病的机制还不清楚。更全面地了解神经元损伤的分子基础对于开发有效治疗传染性海绵状脑病和其他具有相似特征的神经退行性疾病至关重要。本文就线粒体功能障碍和内质网应激介导的神经元死亡的分子机制作一综述，这两种机制在朊病毒疾病的发病机制中起着至关重要的作用。
Transmissible spongiform encephalopathies refer to a group of infectious neurodegenerative diseases with an entirely novel mechanism of transmission and pathophysiology including synaptic damage, dendritic atrophy, vacuolization, and microglial activation. Extensive neuronal loss is the main cause of chronic brain deterioration and fatal outcome of prion diseases. As the final outcome of pathological alterations, neuronal death is a prominent feature of all prion diseases. The mechanisms responsible for prion diseases are not well understood. A more comprehensive understanding of the molecular basis of neuronal damage is essential for the development of an effective therapy for transmissible spongiform encephalopathies and other neurodegenerative diseases sharing similar features. Here, we review the molecular mechanisms of mitochondrial dysfunction and endoplasmic reticulum stress-mediated neuronal death, which play crucial roles in the pathogenisis of prion diseases.