皮质脊柱系统和肌缩侧面硬化症:IFCN手册章节
1Department of Clinical and Movement Sciences, Queen Square Institute of Neurology, UCL, London WC1N 3BG, UK.
概括
灵长类动物的皮层脊柱系统,包括直接的皮层-运动神经元 (CM) 投射,对于熟练的手动是至关重要的. 了解这些途径对于研究像ALS这样的神经退行性疾病至关重要.
科学领域:
- 神经科学是一个神经科学.
- 发动机控制器的控制器
- 灵长类的神经解剖学
背景情况:
- 皮质脊髓系统起源于运动皮质,投射到脊髓网络.
- 一个平行皮质球膜系统的目标是骨运动网络和脑干路径.
- 灵长类的皮层脊髓管具有多种纤维直径,包括快速导电的轴突.
研究的目的:
- 阐明灵长类动物皮质脊柱和皮质运动神经系统 (CM) 的组织和功能.
- 为了突出灵长类动物和非灵长类动物皮质脊柱路径之间的进化差异.
- 为选择合适的动物模型来研究像ALS这样的神经退行性疾病提供信息.
主要方法:
- 在灵长类动物和非灵长类动物中对皮质脊柱和CM系统组织的比较分析.
- 关于这些系统在电机控制中的功能作用的文献综述.
- 检查系统功能障碍与神经退行性疾病的关系.
主要成果:
- 直接CM投射到α运动神经元是灵敏灵长类动物的一个关键特征,起源于"新"M1.
- CM系统参与精细运动控制,特别是远端四肢的分化.
- 灵长类动物和动物之间在皮质脊髓系统组织和功能方面存在显著差异.
结论:
- 在神经退行性疾病研究中,识别灵长类特定的皮质脊髓和CM系统特征对于准确的动物模型选择至关重要.
- 选择性损失快速导电的皮质脊髓轴突和CM连接有助于ALS病理,包括"分裂手"表型和受损的熟练手功能.
- 经过验证的熟练手功能的测试可以作为ALS进展的生物标志物.
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