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Study on mechanisms of autoantibody production in the pathophysiology of lysosomal storage disorders

Research Project

Project/Area Number 20590407
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Experimental pathology
Research InstitutionYokohama City University

Principal Investigator

YAMANAKA Shoji  Yokohama City University, 附属病院, 准教授 (80264604)

Co-Investigator(Kenkyū-buntansha) YAMAGUCHI Akira  横浜市立大学, 医学研究科, 客員研究員 (20381585)
Project Period (FY) 2008 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2010: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2009: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2008: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywordsライソゾーム病 / 自己抗体 / 胸腺 / サイトカイン・ケモカイン
Research Abstract

In this study we focused on thymic event from the point of morphology, thymic subpopulation, and gene expression to see the autoimmune mechanisms happening in Sandhoff disease (SD) mice. Thymus from SD mice greater than 15weeks of age showed marked decrease in the percentage of immature T cells and significantly increased CD4+ T cells. During the involution, apoptotic thymic cells and IgG deposition to T cells were increased. CXCL13, one of these genes, was expressed specifically in the thymus, and B1 cells were increased in the thymus. These results suggest that in SD mice it may convert the usually poorly immunogenic thymus into an organ prone to induce autoimmunity, including chemotaxis of B1 cells toward CXCL13.

Report

(4 results)
  • 2010 Annual Research Report   Final Research Report ( PDF )
  • 2009 Annual Research Report
  • 2008 Annual Research Report
  • Research Products

    (12 results)

All 2010 2009 2008 Other

All Journal Article (4 results) (of which Peer Reviewed: 2 results) Presentation (6 results) Remarks (2 results)

  • [Journal Article] Thymic alterations in GM2 gangliosidoses model mouse.2010

    • Author(s)
      Kanzaki S, Yamaguchi A, Yamaguchi K, Kojima Y, Koumitsu N, Nagashima Y, Nagahama K, Ehara M, Hirayasu Y, Ryo A, Aoki I, Yamanaka S
    • Journal Title

      PLoS One 5(8)

    • Related Report
      2010 Final Research Report
  • [Journal Article] Thymic alterations in GM2 gangliosidoses model mice.2010

    • Author(s)
      Yamanaka S, Yamaguchi A, et al
    • Journal Title

      PLoS One

      Volume: 10

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Involvement of retinal neurons and pigment epithelial cells in a murine model of sandhoff disease.2008

    • Author(s)
      Sango K, Yamanaka S, Ajiki K, Arai N, Takano M.
    • Journal Title

      Ophthalmic Res. 40(5)

      Pages: 241-248

    • Related Report
      2010 Final Research Report
  • [Journal Article] Involvement of retinal neurons and pigment epithelial cells in a murine model of sandhoff disease.2008

    • Author(s)
      Sango K, Yamanaka S, et al
    • Journal Title

      Ophthalmic Res. 40(5)

      Pages: 241-248

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Presentation] Sandhoff病モデルマウス脳におけるα-シヌクレインの病態関与の研究2010

    • Author(s)
      山中正二、山口章
    • Organizer
      第33回日本分子生物学会
    • Place of Presentation
      神戸ポートアイランド(兵庫)
    • Year and Date
      2010-10-07
    • Related Report
      2010 Annual Research Report
  • [Presentation] GM2ガングリオシドーシスにおける末梢免疫システムの異常2009

    • Author(s)
      神崎誠一, 山口章, 山口佳代子, 小島良績, 鈴木京子, 高井俊行, 青木一郎, 山中正二
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(横浜)
    • Year and Date
      2009-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] GM2ガングリオシドーシスの中枢神経系における炎症反応メカニズムの解明2009

    • Author(s)
      山口章, 他
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜
    • Year and Date
      2009-12-10
    • Related Report
      2009 Annual Research Report
  • [Presentation] M2ガングリオシドーシスにおける末梢免疫システムの異常2009

    • Author(s)
      神崎誠一, 他
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜
    • Year and Date
      2009-12-10
    • Related Report
      2009 Annual Research Report
  • [Presentation] サンドホフ病モデルマウスを用いた網羅的サイトカインの解析2008

    • Author(s)
      幸光範子, 神崎誠一, 山口章, 山中正二, 青木一郎
    • Organizer
      第97回日本病理学会総会
    • Place of Presentation
      全日空ホテル金沢(金沢)
    • Year and Date
      2008-05-16
    • Related Report
      2010 Final Research Report
  • [Presentation] サンドホフ病モデルマウスを用いた網羅的サイトカインの解析2008

    • Author(s)
      幸光範子, 山中正二, 他
    • Organizer
      第97回日本病理学会総会
    • Place of Presentation
      金沢
    • Year and Date
      2008-05-16
    • Related Report
      2008 Annual Research Report
  • [Remarks] ホームページ等

    • URL

      http://www-user.yokohama-cu.ac.jp/~byori2/index.html

    • Related Report
      2010 Final Research Report
  • [Remarks]

    • URL

      http://www-user.yokohama-cu.ac.jp/~byori2/index.html

    • Related Report
      2010 Annual Research Report

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Published: 2008-04-01   Modified: 2016-04-21  

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