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Author:
Date:
2014-03-20
[Abstract] Prion diseases are transmissible, fatal, neurodegenerative diseases in human and animals. The molecular basis of neurodegeneration in prion diseases is largely unclear. Developing a cellular model capable of monitoring prion-induced cytotoxicity would be a promising approach for better understanding the prion pathogenesis. One candidate cellular assay is a model based on neurospheres, which contains neural stem cells (NSCs). Both undifferentiated and differentiated NSCs have been demonstrated to be permissive to prion infection, and prion-induced cytopathic changes in differentiated ...
[摘要] 朊病毒疾病是人类和动物中可传播的，致命的，神经变性疾病。 朊病毒疾病中神经变性的分子基础很大程度上不清楚。 开发能够监测朊病毒诱导的细胞毒性的细胞模型将是更好地理解朊病毒发病机制的有前途的方法。 一种候选细胞测定是基于神经球的模型，其含有神经干细胞（NSC）。 未分化和分化的NSCs都被证明是容许朊病毒感染，并且报道了分化的神经球培养物中的朊病毒诱导的细胞病变变化（Iwamaru等人，2013）。 该协议描述了诱导NSCs从过度表达朊病毒蛋白（tga20小鼠）的转基因小鼠分化成易感染朊病毒感染的培养物的过程。
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