Molecular pathomechanisms and gene/protein/regeneration therapy for amyotrophic lateral sclerosis

肌萎缩侧索硬化症的分子病理机制和基因/蛋白质/再生治疗

• 批准号: 21390267
• 负责人: ABE Koji
• 金额: $ 10.65万
• 依托单位: Okayama University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (B)
• 财政年份: 2009
• 资助国家: 日本
• 起止时间: 2009 至 2011
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21390267/
• 关键词: 筋萎縮性側索硬化症; ALS; SOD1; neurovascular unit; optineurin; Asidan; SCA36; NOP56; flow-metabolism uncoupling; autophagy; in vivoイメージング; PCAM-1; occludin; collagen IV; トランスジェニックマウス; Nogo-A; NaR; γ1-Laminin; Sema3A

To reveal the molecular pathomechanism of amyotrophic lateral sclerosis(ALS), the respective roles of environmental factors such as extracellular matrix, neurovascular unit, or glucose metabolism around spinal motor neurons were investigated in a G93A-SOD1 transgenic mouse model for ALS. The novel responsible genes for familial ALS(optineurin) and the hereditary motor neuron disease(NOP56) had been discovered from this study, and the specific molecular effects of mutated proteins/transcripts were clarified.

为揭示肌萎缩侧索硬化（ALS）的分子病理机制，在G93 A-SOD 1转基因小鼠模型上研究了脊髓运动神经元周围的细胞外基质、神经血管单位和葡萄糖代谢等环境因素在ALS发病中的作用。本研究发现了家族性ALS（视神经磷酸酶）和遗传性运动神经元病（NOP 56）的新致病基因，阐明了突变蛋白/转录本的特异分子效应。

项目成果

Antiapoptotic and Antiautophagic Effects of Glial Cell Line-Derived Neurotrophic Factor and Hepatocyte Growth Factor After Transient Middle Cerebral Artery Occlusion in Rats

• DOI: 10.1002/jnr.22373
• 发表时间: 2010-08-01
• 期刊: JOURNAL OF NEUROSCIENCE RESEARCH
• 影响因子: 4.2
• 作者: Shang, Jingwei;Deguchi, Kentaro;Abe, Koji
• 通讯作者: Abe, Koji

Spinal anterior horn has the capacity to self‐regenerate in amyotrophic lateral sclerosis model mice

• DOI: 10.1002/jnr.22156
• 发表时间: 2009-12
• 期刊: Journal of Neuroscience Research
• 影响因子: 4.2
• 作者: K. Miyazaki;M. Nagai;N. Morimoto;T. Kurata;Y. Takehisa;Y. Ikeda;K. Abe

Induction of alpha-synuclein, PINK1, DJ-1 in the spinal motor neurons of transgenic mouse carrying a mutant SOD1 gene

携带突变 SOD1 基因的转基因小鼠脊髓运动神经元中 α-突触核蛋白、PINK1、DJ-1 的诱导

• 发表时间: 2010
• 作者: Morimoto N;Miyazaki K;Kurata T;Ikeda Y;Matsuura T;Abe K
• 通讯作者: Abe K

タッチパネル式簡易認知症スクリーニング検査を用いたALS患者認知機能の検討

使用简单的触摸屏痴呆筛查测试检查 ALS 患者的认知功能

• 发表时间: 2011
• 作者: 森本展年;佐藤恒太;倉田智子;池田佳生;松浦徹;阿部康二
• 通讯作者: 阿部康二

Disruption of Neurovascular Unit Prior to Motor Neuron Degeneration in Amyotrophic Lateral Sclerosis

• DOI: 10.1002/jnr.22594
• 发表时间: 2011-05-01
• 期刊: JOURNAL OF NEUROSCIENCE RESEARCH
• 影响因子: 4.2
• 作者: Miyazaki, Kazunori;Ohta, Yasuyuki;Abe, Koji
• 通讯作者: Abe, Koji