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2003 Fiscal Year Final Research Report Summary

Clinical, cell biological and molecular biological research of ALS/parkinsonism-dementia complex of the Kii peninsula of Japan

Research Project

Project/Area Number 13470132
Research Category

Grant-in-Aid for Scientific Research (B)

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

Principal Investigator

KUZUHARRA Shigeki  Mie University, Faculty of Medicine, Professor, 医学部, 教授 (70111383)

Co-Investigator(Kenkyū-buntansha) NARITA Yugo  Mie University, Faculty of Medicine, Associate Professor, 医学部, 助教授 (50242954)
KOKUBO Yasumasa  Mie University, Hospital, Research Associate, 医学部附属病院, 助手 (60263000)
SASAKI Ryogen  Mie University, Faculty of Medicine, Research Associate, 医学部, 助手 (60303723)
Project Period (FY) 2001 – 2003
Keywordsamyotrophic lateral sclerosis / parkinsonism-dementia complex / tauopathy / neurofibrillary tangle / Kii peninsula / Guam island
Research Abstract

(1)Establishment of the disease concept of amyotrophic lateral sclerosis/ parkinsonism- dementia complex of the Kii peninsula of Japan ( Kii ALS/PDC).
We revealed the epidemiology, neurological features, and neurophysiological, neuroradiological and neuropathological findings of Kii ALS/PDC. Kii ALS/PDC was proved to be a tauopathy presenting neurofibrillary tangles (NFTs) in the central nervous system (CNS), which are a specific feature of ALS/PDC in Guam.
(2)Genetic analysis
We are performing a DNA analysis and searching the causative gene of Kii ALS/PDC.
(3)Cell biological study of tau protein
NFTs were not observed in the residents with non-neurodegenerative disease in Kii peninsula, so NFT was revealed to be a specific feature of Kii ALS/PDC.
Tau protein, which is accumulated in the brains of Kii ALS/PDC, was analyzed by immunohistochemical and western blot method.
The isoform. of tau protein of Kii ALS/PDC was composed of 3 and 4 repeat tau. Tau protein was hyper-phospholilated at the same sites of that of Alzheimer's disease.

  • Research Products

    (13 results)

All 2004 2003 2001 2000

All Journal Article (13 results)

  • [Journal Article] Neurofibrillary tangles of residents in ALS and parkinsonism-dementia complex focus in Japan2004

    • Author(s)
      Kokubo Y
    • Journal Title

      Neurology

      Pages: 2399-2401

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Neurofibrillary tangles of residents in ALS and parkinsonism-dementia complex focus in Japan.2004

    • Author(s)
      Yasumasa Kokubo, Shigeki Kuzuhara
    • Journal Title

      Neurology (in press)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Ophthalmomyiasis-like pigmentary retinopathy in ALS/PDC in the Kii peninsula of Japan2003

    • Author(s)
      Kokubo Y
    • Journal Title

      Neurology 60

      Pages: 1725-1726

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Somatosensory evoked potential recovery in Kii amyotrophic lateral sclerosis/parkinsonism dementia complex (Kii ALS/PDC)2003

    • Author(s)
      Machii K
    • Journal Title

      Clin Neurophysiol 114

      Pages: 564-568

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Neuroradiological study on amyotrophic lateral sclerosis and parkinsonism-dementia complex in the Kii peninsula of Japan2003

    • Author(s)
      Kokubo Y
    • Journal Title

      Arch Neurol. 60

      Pages: 1257-1261

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Biochemical and ultrastructural study on neurofibrillary tangles of ALS/PDC cases in the Kii peninsula of Japan2003

    • Author(s)
      Ito N
    • Journal Title

      JNEN 62

      Pages: 791-798

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Ophthalmomyiasis-like pigmentary retinopathy in ALS/PDC in the Kii peninsula of Japan.2003

    • Author(s)
      Yasumasa Kokubo, Shigeki Kuzuhara
    • Journal Title

      Neurology 60

      Pages: 1725-1726

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Somatosensory evoked potential recovery in Kii amyotrophic lateral sclerosis/parkinsonism dementia complex (Kii ALS/PDC).2003

    • Author(s)
      Machii K., Ugawa Y., Kokubo Y., Sasaki R., Kuzuhara S.
    • Journal Title

      Clin Neurophysiol 114

      Pages: 564-568

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Neuroradiological study on amyotrophic lateral sclerosis and parkinsonism-dementia complex in the Kii peninsula of Japan.2003

    • Author(s)
      Yasumasa Kokubo, Shigeki Kuzuhara
    • Journal Title

      Arch Neurol 60

      Pages: 1257-1261

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Biochemical and ultrastructural study on neurofibrillary tangles of ALS/PDC cases in the Kii peninsula of Japan.2003

    • Author(s)
      Noburo Ito, Koichi Ishiguro, Hiroyuki Arai, Yasumasa Kokubo, Ryogen Sasaki, Yugo Narita, Shigeki Kuzuhara
    • Journal Title

      JNEN 62

      Pages: 791-798

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Familial amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Kii peninsula of Japan : Clinical and neuropathological study and Tau analysis2001

    • Author(s)
      Kuzuhara S
    • Journal Title

      Ann Neurol. 49

      Pages: 501-511

    • Description
      「研究成果報告書概要(和文)」より
  • [Journal Article] Familial amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Kii peninsula of Japan : Clinical and neuropathological study and Tau analysis.2001

    • Author(s)
      Shigeki Kuzuhara, Yasumasa Kokubo, Ryogen Sasaki, Yugo Narita, Tadashi Yabana, Masato Hasegawa, Takeshi Iwatsubo
    • Journal Title

      Ann Neurol 49

      Pages: 501-511

    • Description
      「研究成果報告書概要(欧文)」より
  • [Journal Article] Geographical distribution of amyotrophic lateral sclerosis with neurofibrillary tangles in the Kii peninsula of Japan.2000

    • Author(s)
      Yasumasa Kokubo, Shigeki Kuzuhara, Yugo Narita
    • Journal Title

      Journal of Neurology 415

      Pages: 850-852

    • Description
      「研究成果報告書概要(欧文)」より

URL: 

Published: 2006-07-11  

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