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Relation between motor neuronal cell death and persistant sodium current disturbance in ALS

Research Project

Project/Area Number 23591269
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionJuntendo University (2012-2013)
Chiba University (2011)

Principal Investigator

KANAI Kazuaki  順天堂大学, 医学部, 准教授 (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) 2011 – 2013
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2012: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2011: ¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
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 / 神経変性 / カリウムチャネル / 神経保護
Research Abstract

We studied the changes of motor nerve axonal excitabilty properties in motor neuron diseases such as amyotrophic lateral sclerosis (ALS) and spinal and bulbar muscular atrophy (SBMA), and we reported the results of the studies as the published articles.
We analyzed the effects of the changes of motor nerve axonal excitability properties in ALS on the disease prognosis, and we found that motor axonal excitability properties were strong predictors for survival in amyotrophic lateral sclerosis: especially, we clarified that axonal persistent sodium currents, estimated by SDTC and latent addition, are strong and independent predictors for shorter survival in patients with ALS. We reported the results as the publised article in Journal of Neurology, Neurosurgery, and Psychiatry (J Neurol Neurosurg Psychiatry. 2012;83:734-8.).

Report

(4 results)
  • 2013 Annual Research Report   Final Research Report ( PDF )
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (32 results)

All 2014 2013 2012 2011

All Journal Article (19 results) (of which Peer Reviewed: 10 results) Presentation (13 results) (of which Invited: 1 results)

  • [Journal Article] Prominent fatigue in spinal muscular atrophy and spinal and bulbar muscular atrophy : evidence of activity-dependent conduction block2013

    • Author(s)
      Noto Y, Misawa S, Mori M, Kawaguchi N, Kanai K, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Beppu M, Ohmori S, Nakagawa M, Kuwabara S
    • Journal Title

      Clin Neurophysiol

      Volume: 124 Pages: 1893-8

    • Related Report
      2013 Final Research Report
  • [Journal Article] Split hand syndrome in amyotrophic lateral sclerosis : different excitability changes in the thenar and hypothenar motor axons2013

    • Author(s)
      Shibuya K, Misawa S, Nasu S, Sekiguchi Y, Mitsuma S, Beppu M, Ohmori S, Iwai Y, Ito S, Kanai K, Sato Y, Kuwabara S
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 84 Pages: 969-72

    • Related Report
      2013 Final Research Report
  • [Journal Article] Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF722013

    • Author(s)
      Konno T, Shiga A, Tsujino A, Sugai A, Kato T, Kanai K, Yokoseki A, Eguchi H, Kuwabara S, Nishizawa M, Takahashi H, Onodera O
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 84 Pages: 398-401

    • Related Report
      2013 Final Research Report
  • [Journal Article] Mutations in COQ2 in familial and sporadic multiple-system atrophy2013

    • Author(s)
      Multiple-System Atrophy Research Collaboration : Mitsui J, Matsukawa T, Ishiura H, Fukuda Y, Ichikawa Y, Date H, Ahsan B, Nakahara Y, Momose Y, Takahashi Y, Iwata A, Goto J, Yamamoto Y, Komata M, Shirahige K, Hara K, Kakita A, Yamada M, Takahashi H, Onodera O, Nishizawa M, Takashima H, Kuwano R, Watanabe H, Ito M, Sobue G, Soma H, Yabe I, Sasaki H, Aoki M, Ishikawa K, Mizusawa H, Kanai K, Hattori T, Kuwabara S, Arai K, Koyano S, Kuroiwa Y, Hasegawa K, Yuasa T, Yasui K, Nakashima K, Ito H, Izumi Y, Kaji R, Kato T, Kusunoki S, Osaki Y, Horiuchi M, Kondo T, Murayama S, Hattori N, Yamamoto M, Murata M, Satake W, Toda T, Dürr A, Brice A, Filla A, Klockgether T, Wüllner U, Nicholson G, Gilman S, Shults CW, Tanner CM, Kukull WA, Lee VM, Masliah E, Low PA, Sandroni P, Trojanowski JQ, Ozelius L, Foroud T, Tsuji S
    • Journal Title

      N Engl J Med

      Volume: 369 Pages: 233-44

    • Related Report
      2013 Final Research Report
  • [Journal Article] Prominent fatigue in spinal muscular atrophy and spinal and bulbar muscular atrophy: evidence of activity-dependent conduction block.2013

    • Author(s)
      Noto Y, Misawa S, Mori M, Kawaguchi N, Kanai K, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Beppu M, Ohmori S, Nakagawa M, Kuwabara S.
    • Journal Title

      Clin Neurophysiol.

      Volume: 124(9) Issue: 9 Pages: 1893-8

    • DOI

      10.1016/j.clinph.2012.12.053

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Split hand syndrome in amyotrophic lateral sclerosis: different excitability changes in the thenar and hypothenar motor axons2013

    • Author(s)
      Shibuya K
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 印刷中 Issue: 9 Pages: 969-72

    • DOI

      10.1136/jnnp-2012-304109

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.2013

    • Author(s)
      Konno T
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 84(4) Issue: 4 Pages: 398-401

    • DOI

      10.1136/jnnp-2012-302272

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Mutations in COQ2 in familial and sporadic multiple-system atrophy the multiple-system atrophy research collaboration2013

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

      N Engl J Med

      Volume: 369 Issue: 3 Pages: 233

    • DOI

      10.1056/nejmoa1212115

    • URL

      https://localhost/en/publications/228d3923-3adb-4e39-bd77-14bb10709e09

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-432012

    • Author(s)
      Uchida A, Sasaguri H, Kimura N, Tajiri M, Ohkubo T, Ono F, Sakaue F, Kanai K, Hirai T, Sano T, Shibuya K, Kobayashi M, Yamamoto M, Yokota S, Kubodera T, Tomori M, Sakaki K, Enomoto M, Hirai Y, Kumagai J, Yasutomi Y, Mochizuki H, Kuwabara S, Uchihara T, Mizusawa H, Yokota T
    • Journal Title

      Brain

      Volume: 135 Pages: 833-46

    • Related Report
      2013 Final Research Report
  • [Journal Article] Awaji ALS criteria increase the diagnostic sensitivity in patients with bulbar onset2012

    • Author(s)
      Noto Y, Misawa S, Kanai K, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Fujimaki Y, Nakagawa M, Kuwabara S
    • Journal Title

      Clin Neurophysiol

      Volume: 123 Pages: 382-5

    • Related Report
      2013 Final Research Report
  • [Journal Article] Differences in excitability between median and superficial radial sensory axons2012

    • Author(s)
      Fujimaki Y, Kanai K, Misawa S, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Ohmori S, Noto Y, Kugio Y, Shimizu T, Matsubara S, Lin CS, Kuwabara S
    • Journal Title

      Clin Neurophysiol

      Volume: 123 Pages: 1440-5

    • Related Report
      2013 Final Research Report
  • [Journal Article] Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis2012

    • Author(s)
      Kanai K, Shibuya K, Sato Y, Misawa S, Nasu S, Sekiguchi Y, Mitsuma S, Isose S, Fujimaki Y, Ohmori S, Koga S, Kuwabara S
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 83 Pages: 734-8

    • NAID

      130004504845

    • Related Report
      2013 Final Research Report
  • [Journal Article] Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis.2012

    • Author(s)
      Kanai K, Shibuya K, Sato Y, Misawa S, Nasu S, Sekiguchi Y, Mitsuma S, Isose S, Fujimaki Y, Ohmori S, Koga S, Kuwabara S.
    • Journal Title

      J Neurol Neurosurg Psychiatry.

      Volume: 83 Issue: 7 Pages: 734-8

    • DOI

      10.1136/jnnp-2011-301782

    • NAID

      130004504845

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Differences in excitability between median and superficial radial sensory axons2012

    • Author(s)
      Fujimaki Y, Kanai K, Misawa S, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Ohmori S, Noto Y, Kugio Y, Shimizu T, Matsubara S, Lin CS, Kuwabara S
    • Journal Title

      Clin Neurophysiol

      Volume: 123 Issue: 7 Pages: 1440-5

    • DOI

      10.1016/j.clinph.2011.11.002

    • Related Report
      2012 Research-status Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-432012

    • Author(s)
      Uchida A, Sasaguri H, Enomoto M, (18番目), et al
    • Journal Title

      Brain

      Volume: 135 Issue: 3 Pages: 833-46

    • DOI

      10.1093/brain/awr348

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis2012

    • Author(s)
      Kanai K, Shibuya S, Sato Y, Misawa S, Nasu S, Sekiguchi Y, Mitsuma S, Fujimaki F, Ohmori S, Koga S, Kuwabara S
    • Journal Title

      J Neurol Neurosurg Psychiatry.

      Volume: (in press)

    • NAID

      130004504845

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Awaji ALS criteria increase the diagnostic sensitivity in patients with bulbar onset.2012

    • Author(s)
      Noto Y, Misawa S, Kanai K, Shibuya K, Isose S, Nasu S, Sekiguchi Y, Fujimaki Y, Nakagawa M, Kuwabara S.
    • Journal Title

      Clin Neurophysiol.

      Volume: 123 Issue: 2 Pages: 382-5

    • DOI

      10.1016/j.clinph.2011.05.030

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Markedly reduced axonal potassium channel expression in human sporadic amyotrophic lateral sclerosis : an immunohistochemical study2011

    • Author(s)
      Shibuya K, Misawa S, Arai K, Nakata M, Kanai K, Yoshiyama Y, Ito K, Isose S, Noto Y, Nasu S, Sekiguchi Y, Fujimaki Y, Ohmori S, Kitamura H, Sato Y, Kuwabara S
    • Journal Title

      Exp Neurol

      Volume: 232 Pages: 149-53

    • Related Report
      2013 Final Research Report
  • [Journal Article] Markedly reduced axonal potassium channel expression in human sporadic amyotrophic lateral sclerosis : an immunohistochemical study2011

    • Author(s)
      Shibuya K, Misawa S, Arai K, Nakata M, Kanai K, Yoshiyama Y
    • Journal Title

      Exp Neurol

      Volume: 232 Issue: 2 Pages: 149-53

    • DOI

      10.1016/j.expneurol.2011.08.015

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Presentation] The effects of motor axonal potassium currents on the disease progression of amyotrophic lateral sclerosis2014

    • Author(s)
      Kanai K, Yokota T, Shibuya K, Kanouchi T, Iwai Y, Misawa S, Oyama G, Shimo Y, Fujimaki M, Kagamihara Y, Shimizu T, Hattori N, Kuwabara S
    • Organizer
      30^<th> International Congress of Clinical Neurophysiology (ICCN) of the IFCN
    • Place of Presentation
      Berlin
    • Related Report
      2013 Final Research Report
  • [Presentation] The effects of motor axonal potassium currents on the disease progression of amyotrophic lateral sclerosis2014

    • Author(s)
      Kanai K, Yokota T, Shibuya K, Kanouchi T, Iwai Y, Misawa S, Oyama G, Shimo Y, Fujimaki M, Kagamihara Y, Shimizu T, Hattori N, Kuwabara S.
    • Organizer
      30th International Congress on Clinical Neurophysiology (ICCN) of the IFCN
    • Place of Presentation
      Berlin
    • Related Report
      2013 Annual Research Report
  • [Presentation] 軸索持続性Na+電流は筋萎縮性側索硬化症の症状進展速度の規定因子である2013

    • Author(s)
      金井数明、横田隆徳、澁谷和幹、叶内匡、大山彦光、下秦司、藤巻基紀、鏡原康裕、服部信孝、桑原聡
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report
  • [Presentation] 筋萎縮性側索硬化症の症状進展速度予測におけるlatent addition法の有用性2013

    • Author(s)
      金井数明、横田隆徳、澁谷和幹、叶内匡、大山彦光、下秦司、藤巻基紀、三笠道太、鏡原康裕、服部信孝、桑原聡
    • Organizer
      第43回日本臨床神経生理学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report
  • [Presentation] 軸索持続性Na+電流は筋萎縮性側索硬化症の症状進展速度の規定因子である2013

    • Author(s)
      金井 数明、横田 隆徳、澁谷 和幹、叶内 匡、大山 彦光、下 秦司、藤巻 基紀 、鏡原 康裕、服部 信孝、桑原 聡
    • Organizer
      第54回日本神経学会総会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] 筋萎縮性側索硬化症の症状進展速度予測におけるlatent addition法の有用性2013

    • Author(s)
      金井 数明、横田 隆徳、澁谷 和幹、叶内 匡、大山 彦光、下 秦司、藤巻 基紀 、三笠 道太、鏡原 康裕、服部 信孝、桑原 聡
    • Organizer
      第43回日本臨床神経生理学会
    • Place of Presentation
      高知
    • Related Report
      2013 Annual Research Report
  • [Presentation] 長期LH-RHアナログ療法は球脊髄性筋萎縮症の運動神経軸索膜興奮特性異常を改善する2012

    • Author(s)
      金井数明、藤巻由美、能登祐一、山本達也、三澤園子、桑原聡
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report 2012 Research-status Report
  • [Presentation] 軸索興奮特性測定の臨床への貢献(シンポジウム)2012

    • Author(s)
      金井数明
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report
  • [Presentation] 球脊髄性筋萎縮症に対する長期LH-RHアナログ療法における持続性Na+コンダクタンス増大の精密評価2012

    • Author(s)
      金井数明、藤巻由美、能登祐一、山本達也、鵜沢顕之、三澤園子、服部信孝、桑原聡
    • Organizer
      第42回日本臨床神経生理学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report 2012 Research-status Report
  • [Presentation] 軸索興奮特性測定の臨床への貢献2012

    • Author(s)
      金井数明
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] 筋萎縮性側索硬化症の予後予測におけるlatent addition法の有用性2011

    • Author(s)
      金井数明ら
    • Organizer
      第41回日本臨床神経生理学会学術大会
    • Place of Presentation
      静岡
    • Year and Date
      2011-11-12
    • Related Report
      2013 Final Research Report 2011 Research-status Report
  • [Presentation] ALSにおける末梢運動ニューロン軸索興奮性と臨床的意義(シンポジウム)2011

    • Author(s)
      金井数明
    • Organizer
      第41回日本臨床神経生理学会学術大会
    • Place of Presentation
      静岡
    • Year and Date
      2011-11-11
    • Related Report
      2013 Final Research Report
  • [Presentation] ALSにおける末梢運動ニューロン軸索興奮性と臨床的意義(シンポジウム)2011

    • Author(s)
      金井 数明
    • Organizer
      第41回日本臨床神経生理学会学術大会(招待講演)
    • Place of Presentation
      静岡
    • Related Report
      2011 Research-status Report

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Published: 2011-08-05   Modified: 2019-07-29  

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