Pathogenetic basis of seizure susceptibility in a hamster model of cortical malformation

皮质畸形仓鼠模型癫痫易感性的发病机制

• 批准号: 22591125
• 负责人: TAKANO Tomoyuki
• 金额: $ 2.83万
• 依托单位: Shiga University of Medical Science
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2010
• 资助国家: 日本
• 起止时间: 2010 至 2012
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22591125/
• 关键词: 皮質形成異常; 多小脳回症; てんかん; けいれん準備性; 介在神経細胞; てんかん原性; 介在ニューロン; parvalbumin; Ca結合蛋白; 傍多小脳回領域; 大脳皮質奇形; 抑制性神経細胞

Sustained elevation of the intracellular level of Ca2+ in response to glutamate receptor activation can result in depolymerization of microfilaments and microtubules, thus leading to dendritic outgrowth cessation and regression. These processes are suggested to be the primary mechanisms underlying the neuronal migration disorders and the resultant polymicrogyria observed in this experiment. A neuronal tracing study of progenitor cells in the ganglionic eminence using biotinylated dextran amine (BDA) demonstrated the interneurons to be mobilized to the microgyric area out of the ganglionic eminence, thus suggesting the development of new inhibitory neuronal connections after the onset of excitotoxic brain lesions. The lateral paramicrogyral area had the greatest number of parvalbumin-immunoreactive neurons, which was increased significantly in comparison to that in the control cortex (P < 0.01). We suggest that the callosal, thalamic and intracortical afferents to the microgyrus and paramicrogyral area may induce a remarkable imbalance between the excitatory and inhibitory activities of the cortical structures, associated with the epileptogenic mechanism in polymicrogyria.

响应于谷氨酸受体活化的细胞内Ca2+水平的持续升高可导致微丝和微管解聚，从而导致树突状生长停止和消退。这些过程被认为是本实验中观察到的神经元迁移障碍和由此产生的多微脑回的主要机制。使用生物素化葡聚糖胺（BDA）的神经节隆起中祖细胞的神经元示踪研究表明，中间神经元被动员到神经节隆起外的微回区，从而表明兴奋性毒性脑损伤发作后新的抑制性神经元连接的发展。外侧微回旁区的小白蛋白阳性神经元数量最多，与对照区相比，显著增加（P < 0.01）。我们认为，胼胝体，丘脑和皮质内传入的microgyrus和paramicrogyral区可能会导致一个显着的皮质结构之间的兴奋性和抑制性活动的不平衡，与癫痫的发生机制在polymicrogyria。

项目成果

A Japanese patient with Kabuki syndrome and unilateral perisylvian cortical dysplasia

日本一名患有歌舞伎综合征并单侧侧裂周围皮质发育不良的患者

• DOI: 10.1016/j.braindev.2010.04.001
• 发表时间: 2011
• 期刊: Brain Dev
• 作者: Yoshioka S;Takano T;Matsuwake K;Sokoda T;Takeuchi Y
• 通讯作者: Takeuchi Y

Neural Tube Defects - Role of Folate, Prevention Strategies and Genetics

• DOI: 10.5772/1485
• 发表时间: 2012-03
• 作者: K. Narasimhan

Characteristics of the neurological manifestations of paediatric perisylvian polymicrogyria

儿童侧裂周围多小脑回的神经系统表现特征

• 发表时间: 2012
• 作者: Takano T;Ryujin F;Yoshioka S;Sawai C;Sakaue Y;Takeuchi Y
• 通讯作者: Takeuchi Y

ナースの小児科学(改訂5版)

护士儿科（第五修订版）

• 发表时间: 2011
• 作者: 宮原史子;長石純一;林 篤;宮原直樹;西村 玲;花田卓也;村上 潤;岡田晋一;花木啓一;神崎 晋;相原正男;高野知行
• 通讯作者: 高野知行

Polymicrogyria without epilepsy by aberrantly migrating inhibitory interneurons

抑制性中间神经元异常迁移导致不伴癫痫的多小脑回症

• 发表时间: 2010
• 期刊: Epilepsy and Behavior
• 影响因子: 2.6
• 作者: Tomoyuki Takano;et al.
• 通讯作者: et al.