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Involvement in alpha-synuclein function and pathology in lysosomal storage diseases

Research Project

Project/Area Number 25460500
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Experimental pathology
Research InstitutionYokohama City University

Principal Investigator

YAMAGUCHI Akira  横浜市立大学, 医学研究科, 客員講師 (20381585)

Co-Investigator(Kenkyū-buntansha) 山中 正二  横浜市立大学, 附属病院, 准教授 (80264604)
鈴木 京子  横浜市立大学, 医学研究科, 客員研究員 (90420679)
Project Period (FY) 2013-04-01 – 2017-03-31
Project Status Completed (Fiscal Year 2016)
Budget Amount *help
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywordsサンドホフ病 / ライソゾーム蓄積病 / GM2ガングリオシドーシス / α-シヌクレイン / オートファジー / ライソゾーム病 / ガングリオシド / ガングリオシドーシス
Outline of Final Research Achievements

The accumulation of α-synuclein (ASyn) has been observed in several lysosomal storage diseases (LSDs) but it remains unclear if ASyn accumulation contributes to LSD pathology. ASyn also accumulates in the neurons of Sandhoff disease (SD) patients and SD model mice (Hexb-/- ASyn+/+ mice).
In this study, we explored the potential role of ASyn accumulation in the neurodegenerative process of LSDs, we generated Hexb-/- ASyn-/- mice. Here, we present evidence that autophagic and ubiquitin proteasome pathway are impaired, and mitochondrial function are damaged in Hexb-/- ASyn+/+ mice, but this improves in the absence of ASyn. However, this reducing ASyn accumulation is associated with little improvement in any clinical features of Hexb-/- ASyn+/+ mice.

Report

(5 results)
  • 2016 Annual Research Report   Final Research Report ( PDF )
  • 2015 Research-status Report
  • 2014 Research-status Report
  • 2013 Research-status Report
  • Research Products

    (5 results)

All 2016 2015 2014

All Journal Article (1 results) (of which Peer Reviewed: 1 results,  Acknowledgement Compliant: 1 results) Presentation (4 results)

  • [Journal Article] Accumulated α-synuclein affects the progression of GM2 gangliosidoses.2016

    • Author(s)
      Suzuki K, Yamaguchi A, Yamanaka S, Kanzaki S, Kawashima M, Togo T, Katsuse O, Koumitsu N, Aoki N, Iseki E, Kosaka K, Yamaguchi K, Hashimoto M, Aoki I, Hirayasu Y1
    • Journal Title

      Experimental Neurology

      Volume: 284(Part A) Pages: 38-49

    • DOI

      10.1016/j.expneurol.2016.07.011

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Presentation] サンドホフ病におけるB細胞走化性因子CXCL‐13の役割2015

    • Author(s)
      川島真人、山口章、鈴木京子、山中正二、青木一郎
    • Organizer
      第38回日本分子生物学会年会、第88回日本生化学会大会 合同大会
    • Place of Presentation
      神戸ポートアイランド(兵庫県)
    • Year and Date
      2015-12-01
    • Related Report
      2015 Research-status Report
  • [Presentation] αシヌクレインの蓄積がGM2ガングリオシドーシス病に与える影響2015

    • Author(s)
      鈴木京子、山口章、川島真人、神崎誠一、幸光範子、勝瀬大海、青木直哉、都甲崇、南澤麿優覧、青木一郎、橋本款、山中正二、平安良雄
    • Organizer
      第38回日本分子生物学会年会、第88回日本生化学会大会 合同大会
    • Place of Presentation
      神戸ポートアイランド(兵庫県)
    • Year and Date
      2015-12-01
    • Related Report
      2015 Research-status Report
  • [Presentation] サンドホフ病の中枢神経系における免疫異常メカニズムの解明2014

    • Author(s)
      川島真人、山口章、鈴木京子、山中正二、青木一郎
    • Organizer
      第37回分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(神奈川県)
    • Year and Date
      2014-11-25 – 2014-11-27
    • Related Report
      2014 Research-status Report
  • [Presentation] サンドホフ病マウスにおけるα-シヌクレインの病態への関与と機能の研究2014

    • Author(s)
      鈴木京子、山口章、川島真人、神崎誠一、勝瀬大海
    • Organizer
      第87回日本生化学学会大会
    • Place of Presentation
      国立京都国際会館(京都府)
    • Year and Date
      2014-10-15 – 2014-10-18
    • Related Report
      2014 Research-status Report

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Published: 2014-07-25   Modified: 2019-07-29  

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