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The neurophysiological study of the association between K+ channel dysfunction and motor neuronal loss in amyotrophic lateral sclerosis

Research Project

Project/Area Number 20591018
Research Category

Grant-in-Aid for Scientific Research (C)

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

Principal Investigator

KANAI Kazuaki  Chiba University, 医学部附属病院, 助教 (10375751)

Co-Investigator(Kenkyū-buntansha) KUWABARA Satoshi  千葉大学, 大学院・医学研究院, 教授 (70282481)
Project Period (FY) 2008 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2010: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2009: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2008: ¥2,990,000 (Direct Cost: ¥2,300,000、Indirect Cost: ¥690,000)
Keywords筋萎縮性側索硬化症 / チャネロパチー / 神経軸索興奮特性 / ナトリウムチャネル / カリウムチャネル / 神経変性 / 神経保護 / チェネロパチー
Research Abstract

We estimated simultaneously the changes of motor axonal excitability properties and nodal/paranodal ion channel expressions of Na+ and K+ channels in the process of the Wallerian degeneration and regeneration in mice model, and reported the association among them. We also studied the nerve excitability changes in the motor neuron diseases and its associations with the disease pathophysiology, and studied the pathophysiological roles of channelopathy in some neurological disorders. The results were presented in some symposiums and conferences, and were published in some papers.

Report

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

    (48 results)

All 2011 2010 2009 2008

All Journal Article (30 results) (of which Peer Reviewed: 30 results) Presentation (18 results)

  • [Journal Article] Distal motor axonal dysfunction in amyotrophic lateral sclerosis.2011

    • Author(s)
      Noto Y, Kanai K, Misawa S, Kuwabara S (10^<th>).
    • Journal Title

      Journal of the neurological science. 302巻

      Pages: 58-62

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis : specificity, sensitivity, and a possible prognostic value.2011

    • Author(s)
      Noto Y, Shibuya K, Sato Y, Kanai K, Kuwabara S(16^<th>).
    • Journal Title

      Amyotrophic Lateral Sclerosis. 12巻

      Pages: 140-143

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Altered axonal excitability properties in juvenile muscular atrophy on distal upper extremity (Hirayama disease).2011

    • Author(s)
      Sawai S, Misawa S, Kanai K, Kuwabara S(11^<th>).
    • Journal Title

      Clinical Neurophysiology. 122巻

      Pages: 205-209

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study) : a multicentre, randomised, double-blind, placebo-controlled trial.2011

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

      Lancet.Neurol.

      Volume: 9 Pages: 875-884

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Altered axonal excitability properties in juvenile muscular atrophy of distalupper extremity (Hirayama disease).2011

    • Author(s)
      Sawai S, Misawa S, Kanai K.etal.
    • Journal Title

      Clin Neurophysiol.

      Volume: 49 Pages: 205-209

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: specificity, sensitivity, and a possible prognostic value.2011

    • Author(s)
      Noto Y, Shibuya K, Sato Y, Kan ai K., et at
    • Journal Title

      Amyotroph Lateral Scler.

      Volume: 12 Pages: 140-143

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Distal motor axonal dysfunction in amyotrophic lateral sclerosis.2011

    • Author(s)
      Noto Y, Kanai Misawa S, et al.
    • Journal Title

      J Neurol Sci.

      Volume: 49 Pages: 56-62

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study) : a multicentre, randomised, double-blind, placebo-controlled trial.2010

    • Author(s)
      Katsuno M, Kanai K(11^<th>), et al.
    • Journal Title

      Lancet Neurology. 9巻

      Pages: 875-894

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Physicochemical property changes of amino acid residues that accompany missense mutations in SCN1A affect epilepsy phenotype severity.2009

    • Author(s)
      Kanai K, Yoshida S, Hirose S, et al.
    • Journal Title

      Journal of Medical Genetics. 46巻

      Pages: 671-680

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Differences in excitability properties of FDI and ADM motor axons.2009

    • Author(s)
      Bae JS, Sawai S, Misawa S, Kanai K, Isose S, Kuwabara S.
    • Journal Title

      Muscle and Nerve. 39巻

      Pages: 350-354

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Utility of the distal compound muscle action potential duration for diagnosis of demyelinating neuropathies.2009

    • Author(s)
      Isose S, Kuwabara S, Kokubun N, Kanai K (12^<th>), et al.
    • Journal Title

      Journal of peripheral nervous system. 14巻

      Pages: 151-158

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Preserved cardiac (123)I-MIBG uptake and lack of severe autonomic dysfunction in a PARK9 patient.2009

    • Author(s)
      Kanai K, Asahina M, Arai K, Kuwabara S, (9^<th>), et al.
    • Journal Title

      Movement Disorder. 24巻

      Pages: 1403-1404

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Motor nerve hyperexcitability and muscle cramps in Machado-Joseph disease.2009

    • Author(s)
      Kanai K, Kuwabara S.
    • Journal Title

      Archives of Neurology. 139巻

      Pages: 139-139

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Utility of trapezius EMG for diagnosis of amytrophic lateral sclerosis.2009

    • Author(s)
      Sonoo M, Kuwabara S, Shimizu T, et al.
    • Journal Title

      39巻

      Pages: 63-70

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Physicochemical property changes of amino acid residues that accompany missense mutations in SCN1A affect epilepsy phenotype severity.2009

    • Author(s)
      Kanai K, Yoshida S, Hirose S, et al.
    • Journal Title

      J Med Genet. 46

      Pages: 671-679

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Preserved cardiac(123)I-MIBG uptake and lack of severe autonomic dysfunction in a PARK9 patient.2009

    • Author(s)
      Kanai K, Asahina M, Arai K, et al.
    • Journal Title

      Mov Disord. 24

      Pages: 1403-1404

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Utility of the distal compound muscle action potential duration for diag nosis of demyelinating neuropathies.2009

    • Author(s)
      Isose S, Kuwabara S, Kokubun N, et al.
    • Journal Title

      J Peripher Nerv Syst. 14

      Pages: 151-158

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Motor nerve hyperexcitability and muscle cramps in Machado-Joseph disease.2009

    • Author(s)
      Kanai K, Kuwabara S.
    • Journal Title

      Archives of Neurology 66

      Pages: 139-139

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Differences in excitability properties of FDI and ADM motor axons.2009

    • Author(s)
      Bae JS, Sawai S, Misawa S, Kanai K, et al.
    • Journal Title

      Muscle and Nerve 39

      Pages: 350-4

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Changes in excitability properties associated with axonal regeneration in human neuropathy and mouse Wallerian degeneration.2008

    • Author(s)
      Sawai S, Kanai K, Nakata M, Kuwabara S (8^<th>).
    • Journal Title

      Clinical Neurophysiology. 119巻

      Pages: 1097-1105

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Changes in Na(+) channel expression and nodal persistent Na(+) currents associated with peripheral nerve regeneration in mice.2008

    • Author(s)
      Nakata M, Baba H, Kanai K, Kuwabara S (7th).
    • Journal Title

      Muscle and Nerve. 37巻

      Pages: 721-730

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Effects of age on excitability properties in human motor axons.2008

    • Author(s)
      Bae JS, Sawai S, Misawa S, Kanai K, Isose S, Shibuya K, Kuwabara S.
    • Journal Title

      Clinical Neurophysiology. 119巻

      Pages: 2282-2286

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis : frequency, extent, and specificity.2008

    • Author(s)
      Kuwabara S, Sonoo M, Komori T, et al.
    • Journal Title

      Muscle and Nerve. 37巻

      Pages: 426-430

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Genetically confirmed Huntington's disease masquerading as motor neuron disease.2008

    • Author(s)
      Kanai K, Kuwabara S, Sawai S, et al.
    • Journal Title

      Movement Disorder. 23巻

      Pages: 748-751

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] PARK9-linked parkinsonism in eastern Asia : mutation detection in ATP13A2 and clinical phenotype.2008

    • Author(s)
      Ning YP, Kanai K, Tomiyama H, Kuwabara S (9th), et al.
    • Journal Title

      Neurology. 70巻

      Pages: 1491-1493

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Changes in excitability properties associated with axonal regeneration in human neuropathy and mouse Wallerian degeneration.2008

    • Author(s)
      Sawai S, Kanai K, Nakata M, et al
    • Journal Title

      Clinical Neurophysiology 119

      Pages: 1097-105

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Changes in Na(+) channel expression and nodal persistent Na(+)currents associated with peripheral nerve regeneration in mice.2008

    • Author(s)
      Nakata M, Baba H, Kanai K, Et al.
    • Journal Title

      Muscle and Nerve 37

      Pages: 721-30

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Genetically confirmed Huntington' s disease masquerading as motor neuron disease.2008

    • Author(s)
      Kanai K, Kuwabara S, Sawai S, et al.
    • Journal Title

      Movement Disorders 23

      Pages: 748-51

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] PARK9-linked parkinsonism in eastern Asia: mutation detection in ATP13A2 and clinical phenotype.2008

    • Author(s)
      Ning YP, Kanai K, Tomiyama H, et al
    • Journal Title

      Neurology 70

      Pages: 1491-3

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity.2008

    • Author(s)
      Kuwabara S, Sonoo M, Komori T, et al
    • Journal Title

      Muscle and Nerve 37

      Pages: 426-30

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Presentation] 運動神経軸索膜の興奮特性変化はALSにおける独立した予後規定因子である2010

    • Author(s)
      桑原聡、金井数明、渋谷和幹, ら
    • Organizer
      平成22年度難治性疾患克服研究事業神経変性疾患に関する調査研究班班会議
    • Place of Presentation
      東京
    • Year and Date
      2010-12-17
    • Related Report
      2010 Final Research Report
  • [Presentation] 軸索イオンチャネルからみたALSの病態:免疫組織学的検討2010

    • Author(s)
      桑原聡、渋谷和幹、金井数明, ら
    • Organizer
      平成22年度難治性疾患克服研究事業神経変性疾患に関する調査研究班班会議
    • Place of Presentation
      東京
    • Year and Date
      2010-12-17
    • Related Report
      2010 Final Research Report
  • [Presentation] 運動神経軸索膜の興奮特性変化はALSにおける独立した予後規定因子である2010

    • Author(s)
      金井数明、渋谷和幹, ら
    • Organizer
      -平成22年度難治性疾患克服研究事業 神経変性疾患に関する調査研究班班会議
    • Place of Presentation
      東京
    • Year and Date
      2010-12-17
    • Related Report
      2010 Annual Research Report
  • [Presentation] わかりやすい軸索興奮性テスト2010

    • Author(s)
      金井数明
    • Organizer
      第40回日本臨床神経生理学会学術大会<招待講演>
    • Place of Presentation
      神戸
    • Year and Date
      2010-11-01
    • Related Report
      2010 Final Research Report
  • [Presentation] わかりやすい軸索興奮性テスト2010

    • Author(s)
      金井数明
    • Organizer
      第40回日本臨床神経生理学会学術大会
    • Place of Presentation
      神戸
    • Year and Date
      2010-11-01
    • Related Report
      2010 Annual Research Report
  • [Presentation] LH-RH analogue therapy improves the abnormalities of axonal membrane excitability properties in spinal and bulbar muscular atrophy.2010

    • Author(s)
      Kanai K, Shibuya K, Fujimaki Y, et al.
    • Organizer
      29th International Congress of Clinical Neurophysiology
    • Place of Presentation
      神戸
    • Year and Date
      2010-10-29
    • Related Report
      2010 Final Research Report
  • [Presentation] LH-RH analogue therapy improves the abnormalities of axonal membrane excitability properties in spinal and bulbar muscular atrophy2010

    • Author(s)
      Kanai Shibuya K, FujimakiY, et al
    • Organizer
      29th International Congress of Clinical Neurophysiology
    • Place of Presentation
      神戸
    • Year and Date
      2010-10-29
    • Related Report
      2010 Annual Research Report
  • [Presentation] Use of gene expression systems to study cellular effects of human epilepsy mutations.2010

    • Author(s)
      Kanai K
    • Organizer
      The 8th Asian and Oceanian Epilepsy Congress<招待講演>
    • Place of Presentation
      メルボルン(豪州)
    • Year and Date
      2010-10-21
    • Related Report
      2010 Final Research Report
  • [Presentation] Use of gene expression systems to study cellular effects of human epilepsy mutations2010

    • Author(s)
      Kanai K
    • Organizer
      The 8th Asian and Oceanian Epilepsy Congress
    • Place of Presentation
      メルボルン(豪州)
    • Year and Date
      2010-10-21
    • Related Report
      2010 Annual Research Report
  • [Presentation] 軸索興奮性からみた筋萎縮性側索硬化症の病態:"Split hand"の発現機序2009

    • Author(s)
      桑原聡、金井数明、渋谷和幹, ら
    • Organizer
      平成21年度難治性疾患克服研究事業神経変性疾患に関する調査研究班
    • Place of Presentation
      東京
    • Year and Date
      2009-12-18
    • Related Report
      2010 Final Research Report
  • [Presentation] 球脊髄性筋萎縮症と筋萎縮性側索硬化症の運動神経軸索興奮特性の比較2009

    • Author(s)
      金井数明、渋谷和幹、能登祐一, ら
    • Organizer
      第39回日本臨床神経生理学会
    • Place of Presentation
      小倉
    • Year and Date
      2009-11-20
    • Related Report
      2010 Final Research Report 2009 Annual Research Report
  • [Presentation] Transcriptional channelopathyとしての運動ニューロン疾患:神経興奮性異常とイオンチャネル発現のdysregulation2009

    • Author(s)
      金井数明
    • Organizer
      第39回日本臨床神経生理学会<招待講演>
    • Place of Presentation
      小倉
    • Year and Date
      2009-11-20
    • Related Report
      2010 Final Research Report
  • [Presentation] Transcriptional channelopathyとしての運動ニューロン疾患:神経興奮性異常とイオンチャネル発現のdysregulation2009

    • Author(s)
      金井数明
    • Organizer
      第39回日本臨床神経生理学会
    • Place of Presentation
      小倉
    • Year and Date
      2009-11-20
    • Related Report
      2009 Annual Research Report
  • [Presentation] Clinical application of genetic information in epilepsy management and establishment of individualized medicine for epilepsy.2009

    • Author(s)
      Kazuaki Kanai
    • Organizer
      第43回日本てんかん学会<招待講演>
    • Place of Presentation
      弘前
    • Year and Date
      2009-10-23
    • Related Report
      2010 Final Research Report
  • [Presentation] Clinical application of genetic information in epilepsy management and establishment of individualized medicine for epilepsy2009

    • Author(s)
      金井数明
    • Organizer
      第43回日本てんかん学会
    • Place of Presentation
      弘前
    • Year and Date
      2009-10-23
    • Related Report
      2009 Annual Research Report
  • [Presentation] 球脊髄性筋萎縮症における運動神経軸索膜の受動特性2008

    • Author(s)
      金井数明、桑原聡、澤井摂, ら
    • Organizer
      第38回日本臨床神経生理学会総会
    • Place of Presentation
      神戸
    • Year and Date
      2008-11-13
    • Related Report
      2010 Final Research Report 2008 Annual Research Report
  • [Presentation] 球脊髄性筋萎縮症における持続性Na+コンダクタンス上昇2008

    • Author(s)
      金井数明、桑原聡、澤井摂, ら
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-15
    • Related Report
      2010 Final Research Report
  • [Presentation] 球脊髄性筋萎縮症における持続性Na^+コンダクタンス上昇2008

    • Author(s)
      金井数明、桑原聡、澤井摂、ら
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-15
    • Related Report
      2008 Annual Research Report

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

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