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Development of animal model for sporadic ALS

Research Project

Project/Area Number 19390238
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionNagoya University

Principal Investigator

TANAKA Fumiaki  Nagoya University, 大学院・医学系研究科, 准教授 (30378012)

Co-Investigator(Kenkyū-buntansha) SOBUE Gen  名古屋大学, 大学院・医学系研究科, 教授 (20148315)
ADACHI Hiroaki  名古屋大学, 大学院・医学系研究科, COE特任講師 (40432257)
KATSUNO Masahisa  名古屋大学, 高等研究院, 特任講師 (50402566)
IIJIMA Masahiro  名古屋大学, 大学院・医学系研究科, COE特任助教 (40437041)
道勇 学  愛知医科大学, 医学部, 特任教授 (90293703)
Co-Investigator(Renkei-kenkyūsha) DOYU Manabu  愛知医科大学, 医学部, 教授 (90293703)
Project Period (FY) 2007 – 2008
Project Status Completed (Fiscal Year 2008)
Budget Amount *help
¥18,720,000 (Direct Cost: ¥14,400,000、Indirect Cost: ¥4,320,000)
Fiscal Year 2008: ¥7,670,000 (Direct Cost: ¥5,900,000、Indirect Cost: ¥1,770,000)
Fiscal Year 2007: ¥11,050,000 (Direct Cost: ¥8,500,000、Indirect Cost: ¥2,550,000)
KeywordsALS / 運動ニューロン / dynactin1 / cyclin C / 線虫 / 疾患モデル / dynactinl / 遺伝子発現解析
Research Abstract

孤発性ALS患者脊髄運動ニューロンにおいて、神経変性過程の初期より発現が低下しているdynactin1の発現変化を再現する線虫モデルを作成した。このモデルでは、進行性の運動機能障害、運動ニューロン変性を認め、患者運動ニューロンで観察されたcyclin Cの発現増加、核への局在変化を再現していた。これらのことより、dynactin1ノックダウン線虫は孤発性ALSの病態、特に細胞周期関連因子の異常を反映する重要な疾患モデルであると考えられた。

Report

(3 results)
  • 2008 Annual Research Report   Final Research Report ( PDF )
  • 2007 Annual Research Report
  • Research Products

    (37 results)

All 2009 2008 2007

All Journal Article (33 results) (of which Peer Reviewed: 15 results) Presentation (4 results)

  • [Journal Article] The case for cholesterol2009

    • Author(s)
      Katsuno M, Adachi H, Sobue G.
    • Journal Title

      .Nature Med. (in press)

    • Related Report
      2008 Final Research Report
  • [Journal Article] Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis: A study with tractography and diffusion-tensor imaging2009

    • Author(s)
      Senda J, Ito M, Watanabe H, Atsuta N, Kawai Y, Katsuno M, Tanaka F, Naganawa S, Fukatsu H, Sobue G.
    • Journal Title

      Amyotroph Lateral Scler. (in press)

    • Related Report
      2008 Final Research Report
  • [Journal Article] Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk2009

    • Author(s)
      Young JE, Garden GA, Martinez RA, Tanaka F, Sandoval CM, Smith AC, Sopher BL, Lin A, Fischbeck KH, Ellerby LM, Morrison RS, Taylor JP, La Spada AR.
    • Journal Title

      J Neurosci. 29

      Pages: 1987-1997

    • Related Report
      2008 Final Research Report
  • [Journal Article] Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy2009

    • Author(s)
      Banno H, Katsuno M, Suzuki K, Takeuchi Y, Kawashima M, Suga N, Takamori M, Ito M, Nakamura T, Matsuo K, Yamada S, Oki Y, Adachi H, Minamiyama M, Waza M, Atsuta N, Watanabe H, Fujimoto Y, Nakashima T, Tanaka F, Doyu M, Sobue G.
    • Journal Title

      Ann Neurol. 65

      Pages: 140-150

    • Related Report
      2008 Final Research Report
  • [Journal Article] 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse2009

    • Author(s)
      Tokui K, Adachi H, Waza M, Katsuno M, Minamiyama M, Doi H, Tanaka K, Hamazaki J, Murata S, Tanaka F, Sobue G.
    • Journal Title

      Hum Mol Genet 18

      Pages: 898-910

    • Related Report
      2008 Final Research Report
  • [Journal Article] Research Committee on the Neurodegenerative Diseases of Japan. Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis2009

    • Author(s)
      Atsuta N, Watanabe H, Ito M, Tanaka F, Tamakoshi A, Nakano I, Aoki M, Tsuji S, Yuasa T, Takano H, Hayashi H, Kuzuhara S, Sobue G.
    • Journal Title

      J Neurol Sci. 276

      Pages: 163-169

    • Related Report
      2008 Final Research Report
  • [Journal Article] Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy2009

    • Author(s)
      Takeuchi Y, Katsuno M, Banno H, Suzuki K, Kawashima M, Atsuta N, Ito M, Watanabe H, Tanaka F, Sobue G
    • Journal Title

      Muscle Nerve 38

      Pages: 964-971

    • Related Report
      2008 Final Research Report
  • [Journal Article] The case for cholesterol2009

    • Author(s)
      Katsuno M, et al.
    • Journal Title

      Nature Med (印刷中)

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk2009

    • Author(s)
      Young JE, et al.
    • Journal Title

      J Neurosci 29

      Pages: 1987-1997

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy.2009

    • Author(s)
      Banno H, et al.
    • Journal Title

      Ann Neurol 65

      Pages: 140-150

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis : A study with tractography and diffusion-tensor imaging2009

    • Author(s)
      Senda J, et al.
    • Journal Title

      Amyotroph Lateral Scler (印刷中)

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse2009

    • Author(s)
      Tokui K, et al.
    • Journal Title

      Hum Mol Genet 18

      Pages: 898-910

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis2009

    • Author(s)
      Atsuta N, et al.
    • Journal Title

      J Neurol Sci 276

      Pages: 163-169

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A strategy for developing effective amyotropic lateral sclerosis pharmacotherapy: from clinical trials to novel pharmacotherapeutic strategies2008

    • Author(s)
      Yamamoto M, Tanaka F, Tatsumi H, Sobue G.
    • Journal Title

      Expert Opin Pharmacother 9

      Pages: 1845-1857

    • Related Report
      2008 Final Research Report
  • [Journal Article] Molecular genetics and biomarkers of polyglutamine diseases2008

    • Author(s)
      Katsuno M, Banno H, Suzuki K, Takeuchi Y, Kawashima M, Tanaka F, Adachi H, Sobue G.
    • Journal Title

      Curr Mol Med. 8

      Pages: 221-234

    • Related Report
      2008 Final Research Report
  • [Journal Article] CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA2008

    • Author(s)
      Suzuki K, Katsuno M, Banno H, Takeuchi Y, Atsuta N, Ito M, Watanabe H, Yamashita F, Hori N, Nakamura T, Hirayama M, Tanaka F, Sobue G.
    • Journal Title

      Brain 131

      Pages: 229-239

    • Related Report
      2008 Final Research Report
  • [Journal Article] Refractory Peripheral Neuropathy Study Group of Japan. Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese population2008

    • Author(s)
      Iijima M, Koike H, Hattori N, Tamakoshi A, Katsuno M, Tanaka F, Yamamoto M, Arimura K, Sobue G.
    • Journal Title

      J Neurol Neurosurg Psychiatry 79

      Pages: 1040-1043

    • Related Report
      2008 Final Research Report
  • [Journal Article] Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy2008

    • Author(s)
      Takeuchi Y, et al.
    • Journal Title

      Muscle Nerve 38

      Pages: 964-971

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A strategy for developing effective amyotropic lateral sclerosis pharmacotherapy : from clinical trials to novel pharmacotherapeutic strategies2008

    • Author(s)
      Yamamoto M, et al.
    • Journal Title

      Expert Opin Pharmacother 9

      Pages: 1845-1857

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Molecular genetics and biomarkers of polyglutamine diseases2008

    • Author(s)
      Katsuno M, et al.
    • Journal Title

      Curr Mol Med 8

      Pages: 221-234

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA2008

    • Author(s)
      Suzuki K, et al.
    • Journal Title

      Brain 131

      Pages: 229-239

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA.2008

    • Author(s)
      Suzuki K, et. al.
    • Journal Title

      Brain 131

      Pages: 229-239

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis2007

    • Author(s)
      Jiang YM, Yamamoto M, Tanaka F, Ishigaki S, Katsuno M, Adachi H, Niwa J, Doyu M, Yoshida M, Hashizume Y, Sobue G.
    • Journal Title

      J Neuropathol Exp Neurol 66

      Pages: 617-627

    • Related Report
      2008 Final Research Report
  • [Journal Article] CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model2007

    • Author(s)
      Adachi H, Waza M, Tokui K, Katsuno M, Minamiyama M, Tanaka F, Doyu M, Sobue G.
    • Journal Title

      J Neurosci 27

      Pages: 5115-5126

    • Related Report
      2008 Final Research Report
  • [Journal Article] Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD12007

    • Author(s)
      Niwa J, Yamada S, Ishigaki S, Sone J, Takahashi M, Katsuno M, Tanaka F, Doyu M, Sobue G.
    • Journal Title

      J Biol Chem 282

      Pages: 28087-28095

    • Related Report
      2008 Final Research Report
  • [Journal Article] ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor2007

    • Author(s)
      Yang Z, Chang YJ, Yu IC, Yeh S, Wu CC, Miyamoto H, Merry DE, Sobue G, Chen LM, Chang SS, Chang C.
    • Journal Title

      Nauret Med 13

      Pages: 348-353

    • Related Report
      2008 Final Research Report
  • [Journal Article] Scientific highlights. 17th International Symposium on ALS/MND Yokohama, Japan, November 30, 2006 through December 2, 20062007

    • Author(s)
      Sobue G.
    • Journal Title

      Amyotroph Lateral Scler 8

      Pages: 121-123

    • Related Report
      2008 Final Research Report
  • [Journal Article] Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy2007

    • Author(s)
      Adachi H, Waza M, Katsuno M, Tanaka F, Doyu M, Sobue G.
    • Journal Title

      Neuropathol Appl Neurobiol 33

      Pages: 135-151

    • Related Report
      2008 Final Research Report
  • [Journal Article] Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity2007

    • Author(s)
      Ishigaki S, Niwa J, Yamada S, Takahashi M, Ito T, Sone J, Doyu M, Urano F, Sobue G.
    • Journal Title

      Neurobiol Dis 25

      Pages: 331-341

    • Related Report
      2008 Final Research Report
  • [Journal Article] Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis2007

    • Author(s)
      Jiang YM, et. al.
    • Journal Title

      J Neuropathol Exp Neurol 66

      Pages: 617-627

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD12007

    • Author(s)
      Niwa J, et. al.
    • Journal Title

      J Biol Chem 282

      Pages: 28087-28095

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrphy transgenic mouse model2007

    • Author(s)
      Adachi H, et. al.
    • Journal Title

      J Neurosci 27

      Pages: 5115-5126

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity2007

    • Author(s)
      Ishigaki S, et. al.
    • Journal Title

      Neurobiol Dis 25

      Pages: 331-341

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Presentation] 孤発性ALS病態関連分子の探索と疾患モデルの開発2008

    • Author(s)
      田中章景、和座雅浩、丹羽淳一、祖父江 元
    • Organizer
      第49回日本神経学会総会シンポジウム
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-17
    • Related Report
      2008 Final Research Report
  • [Presentation] 孤発性ALS病ALS病態関連分子の探索と疾患モデルの開発2008

    • Author(s)
      田中章景, ら
    • Organizer
      第49回日本神経学会総会シンポジウム
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-17
    • Related Report
      2008 Annual Research Report
  • [Presentation] Dynactin-1ノックダウン線虫モデルの作成2008

    • Author(s)
      和座雅浩、田中章景、蒋月梅、黄哲、勝野雅央、足立弘明、山本正彦、祖父江元
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-16
    • Related Report
      2008 Annual Research Report 2008 Final Research Report
  • [Presentation] 孤発性ALS疾患モデルの開発 : dynactin1ノックダウン細胞の解析2007

    • Author(s)
      田中章景、黄哲、蒋月梅、松尾幸治、和座雅浩、山本正彦、道勇学、祖父江元
    • Organizer
      第48回日本神経学会総会
    • Place of Presentation
      名古屋
    • Year and Date
      2007-05-16
    • Related Report
      2008 Final Research Report

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Published: 2007-04-01   Modified: 2016-04-21  

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