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Determination of abnormal prion protein in peripheral organs of various prion diseases

Research Project

Project/Area Number 17K07097
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionKyushu University

Principal Investigator

Honda Hiroyuki  九州大学, 医学研究院, 助教 (90624057)

Project Period (FY) 2017-04-01 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2019: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2018: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywordsプリオン蛋白 / 下垂体 / 内分泌 / 内分泌臓器 / PK抵抗性 / プリオン / 脳神経疾患 / 病理学 / オリゴマー / 認知症
Outline of Final Research Achievements

Immunohisatochemistry for prion protein (PrP) on some prion diseases (sporadic Creutzfeldt-Jakob diseases and Gerstmann-Strussler-Scheinker diseases) revealed positive findings of PrP in in many adenohypophyseal cells. Proteomics analysis also revealed PrP in pituitary samples. Western blots showed excessive glycosylation was attached to pituitary PrP, and protease-resistant PrP (PrPres) was also detected. In addition, PrP deposition was also identified histologically in the islets of Langerhans, skin, muscle and peripheral nerves. It was suggested that PrP is involved in human endocrine mechanism, and it was also shown that pituitary tissue of prion disease cases contains PrPres and may be a source of infection.

Academic Significance and Societal Importance of the Research Achievements

ヒトの下垂体前葉細胞の細胞質に、多くのプリオン蛋白が含まれており、それらが通常のプリオン蛋白と異なり過剰な糖鎖修飾をされていた。また、プリオン病症例の下垂体組織にはプロテアーゼ抵抗性プリオン蛋白が含まれている事も同時に示した。ヒトの内分泌機構にプリオン蛋白が関与しており、またプリオン病症例の下垂体組織は感染源になりうる可能性がある事を示した。

Report

(4 results)
  • 2019 Annual Research Report   Final Research Report ( PDF )
  • 2018 Research-status Report
  • 2017 Research-status Report
  • Research Products

    (12 results)

All 2020 2019 2018 2017 Other

All Journal Article (7 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 7 results) Presentation (4 results) (of which Int'l Joint Research: 1 results,  Invited: 1 results) Remarks (1 results)

  • [Journal Article] Accumulation of Astrocytic Aquaporin 4 and Aquaporin 1 in Prion Protein Plaques2020

    • Author(s)
      Sadashima Shoko、Honda Hiroyuki、Suzuki Satoshi O、Shijo Masahiro、Aishima Shinichi、Kai Keita、Kira Junichi、Iwaki Toru
    • Journal Title

      Journal of Neuropathology & Experimental Neurology

      Volume: 79 Issue: 4 Pages: 419-429

    • DOI

      10.1093/jnen/nlaa010

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Frequent Detection of Pituitary-Derived PrPres in Human Prion Diseases2019

    • Author(s)
      Honda Hiroyuki、Matsumoto Masaki、Shijo Masahiro、Hamasaki Hideomi、Sadashima Shoko、Suzuki Satoshi O、Aishima Shinichi、Kai Keita、Nakayama Keiichi I、Sasagasako Naokazu、Iwaki Toru
    • Journal Title

      Journal of Neuropathology & Experimental Neurology

      Volume: 78 Issue: 10 Pages: 922-929

    • DOI

      10.1093/jnen/nlz075

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)‐binding protein 1 in Huntington's disease2019

    • Author(s)
      Mori Shinichiro、Honda Hiroyuki、Ishii Takashi、Yoshimura Motoi、Sasagasako Naokazu、Suzuki Satoshi O.、Taniwaki Takayuki、Iwaki Toru
    • Journal Title

      Neuropathology

      Volume: 39 Issue: 5 Pages: 358-367

    • DOI

      10.1111/neup.12600

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Dynactin is involved in Lewy body pathology2018

    • Author(s)
      Chang Shen, Hiroyuki Honda, Satoshi O Suzuki, et al.
    • Journal Title

      Neuropathology

      Volume: 38 Issue: 6 Pages: 583-590

    • DOI

      10.1111/neup.12512

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Mitochondrial dysfunction and altered ribostasis in hippocampal neurons with cytoplasmic inclusions of multiple system atrophy2018

    • Author(s)
      Norihisa Maeda, Hiroyuki Honda, Satoshi O Suzuki, et al.
    • Journal Title

      Neuropathology

      Volume: 38 Issue: 4 Pages: 361-371

    • DOI

      10.1111/neup.12482

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] DCTN1 F52L mutation case of Perry syndrome with progressive supranuclear palsy-like tauopathy.2018

    • Author(s)
      Hiroyuki Honda
    • Journal Title

      Parkinsonism and Related Disorders

      Volume: 印刷中 Pages: 30090-7

    • DOI

      10.1016/j.parkreldis.2018.02.038

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] Different Complicated Brain Pathologies in Monozygotic Twins With Gerstmann-Straussler-Scheinker Disease.2017

    • Author(s)
      Hiroyuki Honda
    • Journal Title

      Journal of Neuropathology & Experimental Neurology

      Volume: 76 Issue: 10 Pages: 654-863

    • DOI

      10.1093/jnen/nlx068

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Presentation] Prion病(教育講演)2020

    • Author(s)
      本田裕之
    • Organizer
      第61回日本神経病理学会総会学術研究会
    • Related Report
      2019 Annual Research Report
    • Invited
  • [Presentation] ヒトPrion病における下垂体由来異常型prion蛋白の検出2019

    • Author(s)
      本田裕之
    • Organizer
      第60回日本神経病理学会総会学術研究会
    • Related Report
      2019 Annual Research Report
  • [Presentation] ヒトprion病における下垂体由来異常型prion蛋白の検出2019

    • Author(s)
      本田裕之、松本雅紀、濱崎英臣
    • Organizer
      日本神経病理学会
    • Related Report
      2018 Research-status Report
  • [Presentation] Dynactin is involved in Lewy body pathology2018

    • Author(s)
      Hiroyuki Honda, Chang Shen, Satoshi O Suzuki, et al.
    • Organizer
      日本神経病理学会
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research
  • [Remarks] 九州大学研究者情報 本田裕之

    • URL

      https://hyoka.ofc.kyushu-u.ac.jp/search/details/K004446/research.html

    • Related Report
      2019 Annual Research Report

URL: 

Published: 2017-04-28   Modified: 2021-02-19  

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