Elucidation of mechanisms for toxicity of the protein aggregation in amyotrophic lateral sclerosis

Research Project

Project/Area Number	20800076
Research Category	Grant-in-Aid for Young Scientists (Start-up)
Allocation Type	Single-year Grants
Research Field	Neuroscience in general
Research Institution	The Institute of Physical and Chemical Research
Principal Investigator	INOUE Shinichi The Institute of Physical and Chemical Research, 田中研究ユニット, 研究員 (20466030)
Project Period (FY)	2008 – 2009
Project Status	Completed (Fiscal Year 2009)
Budget Amount *help	¥3,302,000 (Direct Cost: ¥2,540,000、Indirect Cost: ¥762,000) Fiscal Year 2009: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000) Fiscal Year 2008: ¥1,742,000 (Direct Cost: ¥1,340,000、Indirect Cost: ¥402,000)
Keywords	筋萎縮性側索硬化症 / アミロイド線維 / ミトコンドリア / 脂質 / 変異型SOD1 / 蛋白質凝集体 / ALS
Research Abstract	Here we show that mutant human SOD1 proteins formed amyloid fibril, and possessed a seeding ability. Mitochondria-specific lipid inhibit amyloid fibril aggregation, rather than promote it. Furthermore, this study confirmed toxicity of the mutant SOD1 amyloid fibrils.

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