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Elucidation of the prion pathogenic decision mechanism through a copper ion binding site of a prion protein

Research Project

Project/Area Number 25870479
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Veterinary medical science
Research InstitutionThe University of Tokushima

Principal Investigator

HARA Hideyuki  徳島大学, 疾患酵素学研究センター, 助教 (40469953)

Project Period (FY) 2013-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2013: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
Keywordsプリオン / 構造変換 / 銅イオン / オクタペプチドリピート / OR / リピート部位 / 銅 / 神経変性 / クロイツフェルト・ヤコブ病 / 狂牛病 / スクレイピー
Outline of Final Research Achievements

Prion diseases are characterized by the replicative propagation of disease-associated forms of prion protein (PrPSc; PrP refers to prion protein). Structural conversion of the normal cellular isoform, designated PrPC, into PrPSc is a key event in prion propagation. However, the conversion mechanism remains unclear. In this study, we intracerebrally inoculated prions into tg(PrPΔOR)/Prnp0/0 mice, which express mouse PrP missing only the octapeptide repeat (OR) region on the PrP-null background. There are different incubation time in infected the prions. As a result, the mechanism of structural conversion was depending on the type of prions. Also we examined the effects of copper ions on the pathological progression of prion disease. However, copper ions didn't have any influence on the pathological progression.

Report

(3 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • Research Products

    (5 results)

All 2014 2013

All Journal Article (3 results) (of which Peer Reviewed: 2 results,  Open Access: 1 results) Presentation (2 results)

  • [Journal Article] Mouse-hamster chimeric prion protein (PrP) devoid of N-terminal residues 23-88 restores susceptibility to 22L prions, but not to RML prions in PrP-knockout mice.2014

    • Author(s)
      Uchiyama K, Miyata H, Yano M, Yamaguchi Y, Imamura M, Muramatsu N, Das NR, Chida J, Hara H, Sakaguchi S.
    • Journal Title

      PLoS One

      Volume: 9 Issue: 10 Pages: e109737-e109737

    • DOI

      10.1371/journal.pone.0109737

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Species-barrier phenomenon in prion transmissibility from a viewpoint of protein science2013

    • Author(s)
      Hagiwara, K., Hara, H., and Hanada, K.
    • Journal Title

      J. Biochem.

      Volume: 153 Issue: 2 Pages: 139-145

    • DOI

      10.1093/jb/mvs148

    • NAID

      40019566388

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 「プリオン仮説」の最終証明に挑戦しませんか?2013

    • Author(s)
      原 英之
    • Journal Title

      生物工学会誌

      Volume: 91 Pages: 471-471

    • Related Report
      2013 Research-status Report
  • [Presentation] 超好熱菌由来プロテアーゼによるプリオンタンパク質分解の評価2014

    • Author(s)
      清水 七海、古賀 雄一、作道 章一、原 英之、坂口 末廣、金谷 茂則
    • Organizer
      第87回日本生化学会
    • Place of Presentation
      京都国際会館(京都府・京都市)
    • Year and Date
      2014-10-15 – 2014-10-18
    • Related Report
      2014 Annual Research Report
  • [Presentation] 超好熱菌由来プロテアーゼによるプリオンタンパク質分解の評価2014

    • Author(s)
      古賀 雄一、清水 七海、作道 章一、原 英之、坂口 末廣、金谷 茂則
    • Organizer
      第66回日本生物工学会大会,
    • Place of Presentation
      札幌コンベンションセンター(北海道・札幌市)
    • Year and Date
      2014-09-09 – 2014-09-11
    • Related Report
      2014 Annual Research Report

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Published: 2014-07-25   Modified: 2019-07-29  

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