Analysis of protein aggregation in amyotrophic lateral sclerosis.

• Project/Area Number: 21790275
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Single-year Grants
• Research Field: General medical chemistry
• Research Institution: Osaka University
• Principal Investigator: MATSUMOTO Ayako Osaka University, 産業科学研究所, 特任助教(常勤) (60444519)
• Project Period (FY): 2009 – 2010
• Project Status: Completed (Fiscal Year 2010)
• Budget Amount: ¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000); Fiscal Year 2010: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2009: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
• Keywords: スーパーオキシドジスムターゼ / 筋萎縮性側索硬化症 / 凝集体 / 筋萎縮性硬化症 / SOD / オリゴマー / 酵素 / 蛋白質 / 脳神経疾患

Research Abstract

We have explored a mechanism of protein aggregation in amyotrophic lateral sclerosis and found that apo SOD1 and FALS mutant SODs become a substrate for transglutaminase. We could detect soluble oligomeric SODls by high-resolution clear native polyacrylamide gel electrophoresis (hrCN-PAGE). Moreover, hrCN-PAGE can be followed by in-gel activity assay, which revealed enzymatic activities on some oligomeric SODIs from both wild type and FALS mutants.

Research Products

• [Presentation] 筋萎縮性側索硬化症(ALS)の凝集体形成におけるトランスグルタミナーゼの関与 (2012)
  • Author(s): 松本紋子・松本明郎・谷口直之
  • Organizer: トランスグルタミナーゼ研究会
  • Place of Presentation: 神戸
  • Year and Date: 2012-12-08
• [Presentation] 筋萎縮性側索硬化症(ALS)の凝集体形成におけるトランスグルタミナーゼの関与 (2010)
  • Author(s): 松本紋子、松本明郎、谷口直之
  • Organizer: 第13回トランスグルタミナーゼ研究会学術集会
  • Place of Presentation: 神戸
  • Year and Date: 2010-12-08
• [Presentation] Improved analysis for the oligomeric SOD1s of familial amyotrophic lateral sclerosis and their SOD activities by high resolutionclearnativeelectrophoresis (2010)
  • Author(s): 松本紋子・松本明郎、谷口直之
  • Organizer: 第33回日本分子生物学会第83回日本生化学会大会合同大会(BMB2010)
  • Place of Presentation: 神戸
  • Year and Date: 2010-12-07
• [Presentation] Improved analysis for the oligomeric SOD1s of familial amyotrophic lateral sclerosis and their SOD activities by high resolution clear native electrophoresis (2010)
  • Author(s): 松本紋子、松本明郎、谷口直之
  • Organizer: BMB2010(第33回日本分子生物学会年会、第83回日本生化学会大会合同大会)
  • Place of Presentation: 神戸
• [Book] ALS における酸化ストレスおよび酸化型SOD1の関与(監修:谷口直之、編集:赤池孝章、鈴木敬一郎、内田浩二)(病態解明に迫る活性酸素シグナルと酸化ストレス、分担執筆) (2009)
  • Author(s): 藤原範子・松本紋子・谷口直之・鈴木敬一郎
  • Publisher: 羊土社