The study of the molecular mechanism of polyglutamine disease model mice and search for new therapeutic molecule

多聚谷氨酰胺疾病模型小鼠分子机制研究及寻找新的治疗分子

• 批准号: 21790845
• 负责人: SAKAE Nobutaka
• 金额: $ 2.75万
• 依托单位: Kyushu University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Young Scientists (B)
• 财政年份: 2009
• 资助国家: 日本
• 起止时间: 2009 至 2010
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21790845/
• 关键词: 神経分子病態学; 脊髄小脳変性症; ポリグルタミン病; モデルマウス; 細胞内輸送機能; ゴルジ体; 脳神経疾患; 細胞内輸送機能異常; ユビキチン・プロテアソーム; 神経化学; 神経病理学

Using the expanded 155 polyglutamine overexpression transgenic mice, we analyzed the molecular mechanism of the mice from the early to end stage of the clinical course by sequential western blot analysis and immunofluorescence study. We revealed that the fragmentation of Golgi apparatus were occurred in early stage of the Tg mice. Further, we identified one of the Golgi related molecule was increased in protein expression level and the localization was different compared to the littermate NT mice at early stage.

利用扩增的155只多聚谷氨酰胺过表达转基因小鼠，通过序贯免疫印迹分析和免疫荧光研究，分析了小鼠从临床病程早期到末期的分子机制。我们发现高尔基体的破碎发生在Tg小鼠的早期阶段。此外，我们发现与早期同窝NT小鼠相比，一种高尔基体相关分子的蛋白质表达水平增加，并且定位不同。

项目成果

Phenotypic spectrum of hereditary neuralgic amyotrophy caused by the SEPT9 R88W mutation

• DOI: 10.1136/jnnp.2008.168260
• 发表时间: 2010-01-10
• 期刊: JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
• 影响因子: 11
• 作者: Ueda, M.;Kawamura, N.;Kira, J-i
• 通讯作者: Kira, J-i

Analysis of the MJD/SCA3 Tg mice from the view of neuronal dysfunction

从神经元功能障碍角度分析MJD/SCA3 Tg小鼠

• 发表时间: 2010
• 作者: Ueda M;Kawamura N;Tateishi T;Sakae N;Motomura K;Ohyaei Y;et al;栄信孝
• 通讯作者: 栄信孝

Increase in p53 protein levels by presenilin 1 gene mutations and its inhibition by secretase inhibitors.

• DOI: 10.3233/jad-2009-0990
• 发表时间: 2009
• 期刊: Journal of Alzheimer's disease : JAD
• 作者: Linqing Ma;Y. Ohyagi;Katsue Miyoshi;N. Sakae;K. Motomura;T. Taniwaki;H. Furuya;K. Takeda;T. Tabira;J. Kira

運動失調症における細胞内輸送機能の検討

共济失调细胞内转运功能的检查

• 发表时间: 2010
• 作者: Ueda M;Kawamura N;Tateishi T;Sakae N;Motomura K;Ohyaei Y;et al;栄信孝;栄信孝
• 通讯作者: 栄信孝

A study of the mechanism of spinocerebellar ataxia from the view of neuronal dysfunction

从神经元功能障碍角度探讨脊髓小脑共济失调的机制

• 发表时间: 2009
• 作者: 栄信孝;大八木保政;吉良潤一
• 通讯作者: 吉良潤一