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Biochemical analysis of abnormal prion protein in the cases with sporadic Creutzfeldt-Jakob disease.

Research Project

Project/Area Number 17500232
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionKYUSHU UNIVERSITY

Principal Investigator

SASAKI Kensuke  Kyushu University, Graduate School of Medical Sciences, Research Associate, 大学院医学研究院, 助手 (80380616)

Co-Investigator(Kenkyū-buntansha) IWAKI Toru  Kyushu University, Graduate School of Medical Sciences, Professor, 大学院医学研究院, 教授 (40221098)
Project Period (FY) 2005 – 2006
Project Status Completed (Fiscal Year 2006)
Budget Amount *help
¥3,600,000 (Direct Cost: ¥3,600,000)
Fiscal Year 2006: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2005: ¥2,600,000 (Direct Cost: ¥2,600,000)
Keywordsprion disease / prion protein types / immunoblot / oligomer / Creutzfeldt-Jakob disease / 凝集体 / 遠心カラム / 孤発性ヤコブ病 / プリオン蛋白 / 電気泳動
Research Abstract

We could examine one case with sporadic Creutzfeldt-Jakob disease (CJD) in Japan that expressed co-occurrence of type 1 and type 2 protease-resistant prion protein (PrP). Pathological features of the unique case showed both synaptic and perivacuolar PrP depositions. Coexistence of type 1 and type 2 PrP was observed in only single case (4.5%) among 22 cases examined. We have examined PrP types in sporadic CJD cases in United Kingdom and found that 7 (38.9%) out of 18 cases showed coexistence of type 1 and type 2 PrP, which was significantly higher level than in Japan. Genetic differences among races were also elucidated as reported previously ; codon 129 polymorphism of the prion protein gene consisted entirely of methionin homozygosity in all Japanese cases (100%), in contrast with UK cases (55.6%). Although the pathological grade of pontine nuclei varied among cases, the severity was not affected by the difference of pathogen strain, but merely by the time course of disease progression. Homogeneity of PrP types and clinicopathological features in Japanese sporadic CJD cases may be based on the ethnic background in part.
Because the biochemical properties of protease-resistant PrP in Japanese sporadic CJD cases were relatively homogeneous, we tried to detect PrP oligomers so that we could classify the abnormal PrP based on the conformational differences. We determined an alternative method, which would be a simple and riskless procedure to detect PrP aggregates. The optimized condition was established to solubilize cellular PrP as monomers and leave abnormal PrP as aggregates or oligomers. Therefore we could separate PrP aggregates by the simple method in a shorter time. Recently it has been found that PrP oligomers rather than highly aggregated PrP fibrils have more potent infectivity and neurotoxicity. This simple method for PrP oligomer detection may be suitable for practical use for the analysis of prion diseases.

Report

(3 results)
  • 2006 Annual Research Report   Final Research Report Summary
  • 2005 Annual Research Report
  • Research Products

    (15 results)

All 2007 2006 2005

All Journal Article (15 results)

  • [Journal Article] Altered expression of COX-2 in subdivisions of the hippocampus during aging and in Alzheimer's disease : the Hisayama study.2007

    • Author(s)
      Fujimi K
    • Journal Title

      Dementia Geriatric Cogn. Dis. 23

      Pages: 323-331

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Altered expression of COX-2 in subdivisions of the hippocampus during aging and in Alzheimer's disease : the Hisayama study.2007

    • Author(s)
      Fujimi K
    • Journal Title

      Dementia Geriatric Cogn.Dis. 23

      Pages: 323-331

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Altered expression of COX-2 in subdivisions of the hippocampus during aging and in Alzheimer's disease: the Hisayama study.2007

    • Author(s)
      Fujimi K
    • Journal Title

      Dementia Geriatric Cogn. Dis. (in press)

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Clusterin expression in follicular dendritic cells associated with prion protein accumulation.2006

    • Author(s)
      Sasaki K
    • Journal Title

      J. Pathol. 209

      Pages: 484-491

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] Quantitative analysis of neurofibrillary pathology in a general population to reappraise neuropathological criteria for senile dementia of the neurofibrillary tangle type(tangle-only dementia) : The Hisayama study.2006

    • Author(s)
      Noda K
    • Journal Title

      Neuropathology 26

      Pages: 508-518

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Styrylbenzoazole derivatives for imaging of prion plaques and treatment of transmissible spongiform encephalopatheis.2006

    • Author(s)
      Ishikawa K
    • Journal Title

      J. Neurochem. 99

      Pages: 198-205

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] Surface plasmon resonance analysis for the screening of anti-piron compounds.2006

    • Author(s)
      Kawatake S
    • Journal Title

      Biol. Pharm. Bull. 29

      Pages: 927-932

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] CJDの病理学的評価基準.2006

    • Author(s)
      佐々木健介
    • Journal Title

      CLINICAL NEUROSCIENCE 24

      Pages: 327-330

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Clusterin expression in follicular dendritic cells associated with prion protein accumulation.2006

    • Author(s)
      Sasaki K
    • Journal Title

      J.Pathol. 24

      Pages: 327-330

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Styrylbenzoazole derivatives for imaging of prion plaques and treatment of transmissible spongiform encephalopatheis.2006

    • Author(s)
      Ishikawa K
    • Journal Title

      J.Neurochem. 99

      Pages: 198-205

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Surface plasmon resonance analysis for the screening of anti-piron compounds.2006

    • Author(s)
      Kawatake S
    • Journal Title

      Biol.Pharm.Bull. 29

      Pages: 927-932

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] CJDの病理学的評価基準2006

    • Author(s)
      佐々木健介
    • Journal Title

      CLINICAL NEUROSCIENCE 24

      Pages: 327-330

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] Quantitative analysis of neurofibrillary pathology in a general population to reappraise neuropathological criteria for senile dementia of the neurofibrillary tangle type (tangle-only dementia): The Hisayama study.2006

    • Author(s)
      Noda K
    • Journal Title

      Neuropathology 26

      Pages: 508-518

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Quantitative analysis of neurofibrillary pathology in a general population to reappraise neuropathological criteria for senile dementia of the neurofibrillary tangle type (tangle-only dementia) : The Hisayama study.2006

    • Author(s)
      Noda K
    • Journal Title

      Neuropathology (in press)

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Fatal familial insomnia with an unusual prion protein deposition pattern : An autopsy report with an experimental transmission study.2005

    • Author(s)
      Sasaki K
    • Journal Title

      Neuropathol Appl Neurobiol 31

      Pages: 80-87

    • Related Report
      2005 Annual Research Report

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Published: 2005-04-01   Modified: 2016-04-21  

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