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Pathophysiology of amyotrophic lateral ]sclerosi using aipha-tocopherol transfer protein gene knockout mouse.

Research Project

Project/Area Number 13680838
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurochemistry/Neuropharmacology
Research InstitutionTokyo Medical and Dental University

Principal Investigator

INABA Akira  Tokyo Medical and Dental University, Laboratory Meidine, RESEARCH ASSOCIATE, 医学部附属病院, 助手 (10282766)

Co-Investigator(Kenkyū-buntansha) YOKOTA Takanori  Tokyo Medical and Dental University, Neurology, RESEARCH ASSOCIATE, 医学部附属病院, 講師 (90231688)
Project Period (FY) 2001 – 2003
Project Status Completed (Fiscal Year 2003)
Budget Amount *help
¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 2003: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 2002: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 2001: ¥1,300,000 (Direct Cost: ¥1,300,000)
Keywordsvitamin E. / aTTP / ALS. / SOD1 / Oxidative stress / lipid peroxidation / αTTP / SOD1 / 脊髄小脳変性症 / 運動ニューロン疾患 / G93Aトランスジェニックマウス / aTTP KOマウス / SOD / α-tocopherol / 運動ニューロン / 筋萎縮性側索硬化症 / G93A トランスジェニックマウス
Research Abstract

1) MDA and HNE modifications in the brain of aTTP null mouse were increased and improved by vitamin E supplementation.
2) HNE-modified protein is SOD. This modification disturbed the radical scavenge function of SOD, resulting in enhance the oxidative stress by vitamin E deficiency.
3) Dopamine content in the striatum of aTTP null mouse was decreased after administration of MPTP, indicating dopamine decrease in Parkinson disease is related to oxidative stress.
4) Onset of symptom in G93ASOD1 TgM crossed with aTTP null mouse is accelerated with more marked degeneration of anterior horn cell, indicating pathophysiology of ALS is affected by oxidative stress,

Report

(4 results)
  • 2003 Annual Research Report   Final Research Report Summary
  • 2002 Annual Research Report
  • 2001 Annual Research Report
  • Research Products

    (20 results)

All Other

All Publications (20 results)

  • [Publications] Yokota T, et al.: "Delayed-onset ataxia in mice lacking-tocopherol transfer protein : Model for neuronal degeneration caused by chronic oxidative stress."Proc Natl Acad USA. 98. 15185-15190 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Inba A, et al.: "Proximal motor conduction evaluated transcranial magentic stimulation in acquired inflammatory demyelinating neuropathies."Biochem Biophys Res Com. 112. 1936-1945 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Inaba A, et al.: "High voltage electric stimulation of the proximal hypoglossal nerve in normal subjects."Clin Neurophysiol. 113. 925-929 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Inaba A, et al.: "Electrophysiological evaluation of the conduction in the most proximal motor root segment."Muscle and Nerve. 25. 608-611 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Ishibashi S, Yokota T, Inaba A, et al.: "Reversible acute axonal polyneuropathy associted with Wernickle-Korsakoff syndrome : impaired physiological nerve conduction due to thiamine deficiency."J Neurology Neurosurgery and Psychiatry. 74. 674-676 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Inaba A, Yokota T: "Isolated musclocutaneous nerve palsy during sleep."Muscle and Nerve. 28. 773-774 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳: "αtocopherol転移タンパク遺伝子異常によるFriedreich型運動失調症(Ataxia with isokated Vitamin E Deficiency)"内科. 87・4. 686-688 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳: "αtocopherol転移タンパク遺伝子異常によるFriedreich型運動失調症(AVED)の発症機序"神経内科. 57・2. 130-137 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳, 他: "αtocopherol転移タンパク遺伝子欠損マウスにおける遅発性運動失調症"Clinical Neuroscience. 21・2. 177-179 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳: "Friedreich病および類似疾患 -AVED, EAOH-"難病と在宅ケア. 9. 95-98 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横由隆徳: "ビタミンE欠乏症"内科. 94・1. 687-690 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳: "家族性特発性ビタミンE欠乏症の発見と発症機序解明及び治療法の確立"ビタミン. 78・2. 87-92 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 横田隆徳: "RNAiによる神経変性疾患の遺伝子治療をめざして"遺伝子医学. 7・3. 349-354 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Inaba A: "Isolated musculocutaneous nerve palsy during sleep"Muscle and Nerve. 28・6. 773-774 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Ishibashi S: "Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome : impaired physiological nerve conduction due to thiamine deficiency"Journal of Neurology, Neurosurgery and Psyhiatry. 74・5. 674-676 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 横田隆徳: "α-tocopherol転移タンパク遺伝子欠損マウスにおける遅発性運動失調症"CLINICAL NEUROSCIENCE. 21・2. 177-179 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 稲葉彰: "経頭道磁気刺激による皮質延髄路の機能評価"神経内科. (印刷中).

    • Related Report
      2003 Annual Research Report
  • [Publications] 横田隆徳: "αtocopherol転移タンパク遺伝子異常によるFriedreich型運動失調症(AVED)の発症機序"神経内科. 57・2. 130-137 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 横田隆徳, 水澤英洋: "αtocopherol転移タンパク遺伝子欠損マウスにおける遅発性運動失調症"Clinical Neuroscience. 21(2). 177-179 (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Yokota T, Inaba A, et al.: "Delayed-onset ataxia in mice lacking alpha-tocoherol transfer protein : model for neuronal degeneration caused by oxidative stress"Proceedings of the National Academy of Scienceof U.S.A.. 98. 15185-15190 (2001)

    • Related Report
      2001 Annual Research Report

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Published: 2001-04-01   Modified: 2016-04-21  

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