Cortically Evoked Long-Lasting Inhibition of Pallidal Neurons in a Transgenic Mouse Model of Dystonia
Cortically Evoked Long-Lasting Inhibition of Pallidal Neurons in a Transgenic Mouse Model of Dystonia
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DOI：
10.1523/jneurosci.3834-08.2008
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发表时间：
2008-12-17
影响因子：
5.3
通讯作者：
Nambu, Atsushi
中科院分区：
文献类型：
--
作者：
Chiken, Satomi;Shashidharan, Pullanipally;Nambu, Atsushi
肌张力障碍是一种神经疾病，其特征是持续或反复的非随意肌肉收缩和异常姿势。为了了解肌张力障碍的病理生理学机制，对作为DYT1肌张力障碍模型的高动力转基因小鼠进行了神经生理学分析。在这些小鼠中，经常观察到异常的肌肉活动，如激动肌和拮抗肌的共同激活和持续的肌肉激活。清醒状态下的神经元活动记录显示，苍白球外部和内部的自发活动减少，出现爆发和停顿。运动皮质刺激在两个苍白球节段引起由兴奋和随后的长期抑制组成的反应，这在正常小鼠中从未观察到。此外，两个苍白球节的体位排列也是杂乱无章的。苍白球内脑段的皮质输入引起的长期抑制可能会抑制丘脑和皮质的活动，导致在转基因小鼠中观察到的运动亢进。
Dystonia is a neurological disorder characterized by sustained or repetitive involuntary muscle contractions and abnormal postures. To understand the pathophysiology of dystonia, neurophysiological analyses were performed on hyperkinetic transgenic mice generated as a model of DYT1 dystonia. Abnormal muscle activity, such as coactivation of agonist and antagonist muscles and sustained muscle activation, was frequently observed in these mice. Recording of neuronal activity in the awake state revealed reduced spontaneous activity with bursts and pauses in both the external and internal segments of the globus pallidus. Motor cortical stimulation evoked responses composed of excitation and subsequent long-lasting inhibition in both pallidal segments, which were never observed in the normal mice. In addition, the somatotopic arrangements in both pallidal segments were disorganized. Long-lasting inhibition induced by cortical inputs in the internal pallidal segment may disinhibit thalamic and cortical activity, resulting in the motor hyperactivity observed in the transgenic mice.