The primary locus of motor neuron death in an ALS-PDC mouse model.
The primary locus of motor neuron death in an ALS-PDC mouse model.
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DOI：
10.1097/wnr.0b013e32833037ae
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发表时间：
2009-09-23
期刊：
影响因子：
1.7
通讯作者：
Shaw CA
中科院分区：
文献类型：
--
作者：
Lee G;Chu T;Shaw CA
使用基于苏铁种子粉的消耗的肌萎缩性侧索硬化症-帕金森综合征-痴呆综合征的小鼠模型来确定所观察到的运动神经元损失的病理学是否开始于远端轴突或脊髓。在多个时间点进行神经肌肉接头完整性和运动神经元的评估。喂食苏铁颗粒的小鼠在钢丝悬挂上的表现比对照组差。在12周时观察到Cycad喂养的小鼠中的小胶质细胞活化和运动神经元变性，但未观察到反应性星形胶质细胞增殖。在苏铁喂养33周后，运动神经元的损失已经稳定，没有证据表明神经肌肉接头终板去神经支配。这些数据表明，神经元病理开始于索马，并在“死亡向前”模式的远端进行。
A mouse model of amyotrophic lateral sclerosis–parkinsonism–dementia complex based on the consumption of cycad seed flour was used to determine whether the observed pathology of motor neuron loss begins in the distal axons or the spinal cord. Assessments of neuromuscular junction integrity and motor neurons were performed at multiple time points. Mice fed cycad pellets performed worse on the wire hang than controls. Microglial activation in cycad-fed mice was observed with motor neuron degeneration at 12 weeks, but reactive astrocyte proliferation was not observed. After 33 weeks of cycad feeding, motor neuron loss had stabilized, with no evidence of neuromuscular junction endplate denervation. These data suggest that neuronal pathology begins at the soma and proceeds distally in a ‘dying forward’ pattern.