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Amyotrophic lateral sclerosis : the progression mechanism across neuronal junctions

Research Project

Project/Area Number 05680653
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Nerve anatomy/Neuropathology
Research InstitutionNiigata University

Principal Investigator

OYANAGI Kiyomitsu  Niigata University, Brain Research Institute, Associate Professor, 脳研究所, 助教授 (00134958)

Co-Investigator(Kenkyū-buntansha) IKUTA Fusahiro  Niigata University, Brain Research Institute, Professor, 脳研究所, 教授 (20018592)
Project Period (FY) 1993 – 1994
Project Status Completed (Fiscal Year 1994)
Budget Amount *help
¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1994: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 1993: ¥1,100,000 (Direct Cost: ¥1,100,000)
KeywordsAmyotrophic lateral sclerosis / Degeneration / Spinal cord / Anterior horn / Intermediate zone / Intermediolateral nucleus / Transneuronal degeneration / Guam ALS / 運動ニューロン / 肢切断
Research Abstract

Was the degeneration in the extra-motor neuron system of the amyotrophic lateral sclerosis (ALS), induced secondarily to the loss of motor neurons? In relation to this, loss of neurons was found not only in the anterior horn but also in the intermediate zone of the cervical spinal cord in a 56-year-old man who had suffered accidental amputation of the right upper arm 38 years prior to death. This finding indicates that the loss of neurons in the intermediate zone of the spinal cord in ALS patients is sequentially induced at least partly by loss of the anterior horn cells.
The neurons in the intermediolateral nucleus at the thoracic segment of the ALS patients, were involved primarily in the disease process without the formation of Bunina bodies, and were more severely decreased in the respirator-supported patients than non-respirator-supported patients.
We elucidated that the mechanism of neuronal degeneration of Guam ALS is basically different from that of the parkinsonism-dementia complex of Guam, and that Guam ALS occurs initially as classic ALS.

Report

(3 results)
  • 1994 Annual Research Report   Final Research Report Summary
  • 1993 Annual Research Report
  • Research Products

    (46 results)

All Other

All Publications (46 results)

  • [Publications] Takahashi H: "Non-motor neuron system in amyotrophic lateral sclerosis:involvement of Clarke's column and intermediolateral nucleus" Neuropathology. 13. 209-213 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takahashi H: "The intermediolateral nucleus in sporadic amyotrophic lateral sclerosis." Acta Neuropathologica. 86. 190-192 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takahashi H: "Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis." Journal of the Neurological Sciences. 120. 15-21 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Suzuki H: "A quantitative pathological investigation of the cervical cord,roots and ganglia after long-term amputation of the unilateral upper arm." Acta Neuropathologica. 85. 666-673 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 若林孝一: "老年者剖検例におけるレビ-小体の偶発的出現の意義.-加齢およびパーキンソン病との関連について-." 脳と神経. 45. 1033-1038 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takiyama Y: "The gene for Machado-Joseph disease maps to human chromosome 14q" Nature Genetics. 4. 300-304 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takeda S: "Motor neuron disease with multi-system involvement presenting as tetraparesis,Ophthalmoplegia and sensoriautonomic dysfunction." Acta Neuropathologica. 88. 193-200 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Amyotrophic lateral sclerosis of Guam:the nature of the neuropathological findings" Acta Neuropathologica. 88. 405-412 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Topographic investigation of brain atrophy in parkinsonismdementia complex of Guam:a comparison with Alzheimer's disease and progressive supranuclear palsy" Neurodegeneration. 3. 301-304 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "The neostriatum and nucleus accumbens in parkinsonismdementia complex of Guam:a pathological comparison with Alzheimer's disease and progressive supranuclear palsy." Acta Neuropathologica. 88. 122-128 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Rod-like intracytoplasmic inclusions in large neurons of the caudate nucleus:frequent appearance in myotonic dystrophy" Clinical Neuropathology. 13. 134-138 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Wakabayashi K: "Corticobasal degeneration:etiopathological significance of the cytoskeletal alterations." Acta Neuropathologica. 87. 545-553 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 小〓清光: "ヒト線条体および側坐核における神経細胞変性機序に関する観察" 脳と神経. 46. 751-756 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 伊藤拓緯: "頸椎症脊髄症の神経病理学的検討." 日獨医報. 39. 43-51 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 小〓清光: "筋萎縮性側索硬化症の脊髄側索前方部に関する定量的検討" Neuropathology. suppl.14. 153 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 巻渕隆夫: "筋萎縮性側索硬化症脊髄前角シナプスの厚切切片免疫組織化学による検討" Neuropathology. suppl.14. 162 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 小〓清光: "球脊髄性筋萎縮症の脊髄前角病変:ALSとの比較検討" Neuropathology. suppl.14. 165 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 相馬収: "保護蛋白(protective protein)の中枢神経分布とガラクトシアリドーシス" Neuropathology. suppl.14. 192- (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Suzuki H: "Evidence for transneuronal degeneration in the spinal cord in man:a quantitative investigation of neurons in the intermediate zone after long-term amputation of the unilateral upper arm" Acta neuropathologica. (印刷中).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takahashi H: "Non-motor neuron system in amyotrophic lateral sclerosis : involvement of Clarke's column and intermediolateral nucleus" Neuropathology. 13. 209-213 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takahashi H: "The intermediolateral nucleus in sporadic amyotrophic lateral sclerosis" Acta Neuropathologica. 86. 190-192 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takahashi H: "Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis" Journal of the Neurological Sciences. 120. 15-21 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Suzuki H: "A quantitative pathological investigation of the cervical cord, roots and ganglia after long-term amputation of the unilateral upper arm" Acta Neuropathologica. 85. 666-673 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Wakabayashi K: "Incidental occurrence of Lewy bodies in the brains of elderly patients -The relevance to aging and Parkinson's disease-" Brain Nerve. 45. 1033-1038 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takiyama Y: "The gene for Machado-Joseph disease maps to human chromosome 14q" Nature Genetics. 4. 300-304 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Takeda S: "Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction" Acta Neuropathologica. 88. 193-200 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Amyotrophic lateral sclerosis of Guam : the nature of the neuropathological findings" Acta Neuropathologia. 88. 405-412 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Topographic investigation of brain atrophy in parkinsonism-dementia complex of Guam : a comparison with Alzheimer's disease and progressive supranuclear palsy" Neurodegeneration. 3. 301-304 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "The neostriatum and nucleus accumbens in parkinsonism-dementia complex of Guam : a pathological comparison with Alzheimer's disease and progressive supranuclear palsy" Acta Neuropathologica. 88. 122-128 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "Rod-like intracytoplasmic inclusions in large neurons of the caudate nucleus : frequent appearance in myotonic dystrophy" Clinical Neuropathology. 13. 134-138 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Wakabayashi K: "Corticobasal degeneration : etiopathological significance of the cytoskeletal alterations" Acta Neuropathologica. 87. 545-553 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "On the degenerating process of the neurons in the human neostriatum and nucleus accumbens" Brain Nerve. 46. 751-756 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Ito T: "Neuropathology of cervical spondylotic myelopathies" Nichi-Doku Iho. 39. 43-51 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "The anterolateral funiculus in the spinal cord in amyotrophic lateral sclerosis" Neuropathology Suppl. 14. 153 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Makifuchi T: "Synapses in the anterior horn of amyotrophic lateral sclerosis : immunohistochemical examination using thick spinal cord sections" Neuropathology. Suppl.14. 162 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Oyanagi K: "The rough endoplasmic reticulum in the anterior horn cells of bulbospinal muscular atrophy ultrastructural comparison with amyotrophic lateral sclerosis" Neuropathology. Suppl.14. 165 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Sohma O: "Immunohistochemical analysis of the protective protein in tissues from normal individuals and a patient with galactosialidosis." Neuropathology. Suppl.14. 192 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Suzuki H: "Evidence for transneuronal degeneration in the spinal cord in man : aquantitative investigation of neurons in the intermediate zone after long-term amputation of the unilateral upper arm" Acta neuropathologica. (in press).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Suzuki Hiroshi: "Evidence for transneuronal degeneration in the spinal cord in map: a quantitative investigation of neurons in the intermediate zone after long-term amputation of the unilateral upper arm" Acta Neuropathologica. (印刷中). (1995)

    • Related Report
      1994 Annual Research Report
  • [Publications] Takeda,Shigeki: "Motor neuron disease with multi-system involvement presenting as tetraparesis,ophthalmoplegia and sensori-autonomic dysfunction" Acta Neuropathologica. 88. 193-200 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] Oyanagi,Kiyomitsu: "Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological findings" Acta Neuropathologica. 88. 405-412 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 伊藤拓緯: "頸椎症性脊髄症の神経病理学的検討" 日獨医報. 39. 43-51 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] Wakabayashi,Koichi: "Corticobasal degeneration:etiopathologic significance of the cytoskeletal alterations" Acta Neuropathologica. (印刷中). (1994)

    • Related Report
      1993 Annual Research Report
  • [Publications] Suzuki,Hiroshi: "A quantitative pathological investigation of the cervical cord,roots and ganglia after long-term amputation of the unilateral upper arm" Acta Neuropathologica. 85. 666-673 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Takahashi,Hitoshi: "The intermediolateral nucleus in sporadic amyotrophic lateral sclerosis" Acta Neuropathologica. 86. 190-192 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Takahashi,Hitoshi: "Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis" Journal of the Neurological Sciences. 120. 15-21 (1993)

    • Related Report
      1993 Annual Research Report

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Published: 1993-04-01   Modified: 2016-04-21  

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