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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
Project Status Completed (Fiscal Year 2003)
Budget Amount *help
¥14,300,000 (Direct Cost: ¥14,300,000)
Fiscal Year 2003: ¥2,800,000 (Direct Cost: ¥2,800,000)
Fiscal Year 2002: ¥1,900,000 (Direct Cost: ¥1,900,000)
Fiscal Year 2001: ¥9,600,000 (Direct Cost: ¥9,600,000)
Keywordsamyotrophic lateral sclerosis / parkinsonism-dementia complex / tauopathy / neurofibrillary tangle / Kii peninsula / Guam island / 神経難病 / タウ蛋白 / パーキンソニズム / 痴呆
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.

Report

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

    (29 results)

All 2004 2003 2001 2000 Other

All Journal Article (13 results) Publications (16 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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [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
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Kokubo Y: "Ophthalmomyiasis-like pigmentary retinopathy in ALS/PDC in the Kii peninsula of Japan."Neurology. 60. 1725-1726 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Machii K: "Somatosensory evoked potential recovery in Kii amyotrophic lateral sclerosis/parkinsonism dementia complex (Kii ALS/PDC)"Clin Neurophysiol. 114. 564-568 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Kokubo Y: "Neuroradiological study on amyotrophic lateral sclerosis and parkinsonism-dementia complex in the Kii peninsula of Japan"Arch Neurol.. 60. 1257-1261 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Ito N: "Biochemical and ultrastructural study on neurofibrillary tangles of ALS/PDC cases in the Kii peninsula of Japan"JNEN. 62. 791-798 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 葛原茂樹: "紀伊半島のALS-parkinsonism-dementia complex"Dementia Japan. 16. 173-128 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Saito y: "Early-onset, rapidly progressive familial fauopathy with R406W mutation"Neurology. 58. 811-813 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 葛原茂樹: "前頭側頭葉型痴呆-特集・アルツハイマー病"総合臨牀. 51. 88-94 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 葛原茂樹: "アルツハイマー病.治療と早期診断に関する最近の話題"成人病と生活習慣病. 32. 623-628 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Machii K: "Somatosensory evoked potentioal recovery in Kii amyotrophic lateral sclerosis/parkinsonism dementia complex (Kii ALS/PDC)"Clin Neurophysiol. (in press). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Kokubo Y: "Ophthalmomyiasis-like pigmentary retinopathy in ALS/PDC in the Kii peninsula of Japan"Neurology. (inpress). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] Kuzuhara S: "Familial amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Kii peninsula of Japan:clinical and neuropathological study and tau analysis"Ann Neurol. 49. 501-511 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 葛原茂樹: "紀伊半島のALSの疫学"神経内科. 54. 13-19 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 葛原茂樹: "Frontotemporal dementia概説"神経内科. 55. 103-110 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 小久保康昌: "紀伊半島多発地域の筋萎縮性側索硬化症とパーキンソン痴呆複合の臨床神経学的および神経病理学的検討"臨床神経. (発表予定). (2002)

    • Related Report
      2001 Annual Research Report
  • [Publications] Ito N: "Large-scate,multicinter study of cerebrospinal fluid tau protein phosphorylated at serine 199 for the antemortem diagnosis of Alzhelmer's disease"Ann Neurol. 50. 150-156 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Kuzuhara S: "Neuroscientific basis of dementia"Amyotrophic lateral sclerosis/parldnsonism-dememtia complex of the Kii peninsula of Japan(Kii Als/Pdc)may be a famillal tauopathy.Epidemlological trends,clinical features,neuropathology and molecular genetics. 9 (2001)

    • Related Report
      2001 Annual Research Report

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

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