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Basic research on establishment, maintenance, and therapy of non-human primate model of mucopolysaccharidosis

Research Project

Project/Area Number 18K06442
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 45050:Physical anthropology-related
Research InstitutionKyoto University

Principal Investigator

Oishi Takao  京都大学, 霊長類研究所, 准教授 (40346036)

Project Period (FY) 2018-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2020: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2019: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2018: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Keywordsライソゾーム病 / 疾患モデル / ニホンザル / ムコ多糖症 / ハーラー病 / 家系解析 / 霊長類 / 繁殖コロニー / 酵素補充療法 / シャイエ病 / 自然発症 / 遺伝病モデル / 水頭症 / モデル動物 / 疾病モデル動物 / 中枢神経機能
Outline of Final Research Achievements

A missense mutation was found in alpha-L-iduronidase, which results in mucopolysaccharidosis type I (MPSI), among the Japanese monkeys kept in a colony of Primate Research Institute, Kyoto University. Four monkeys were homozygous and showed MPSI symptoms. Some others were heterozygous and showed no symptoms. It is necessary to establish an effective production system of MPSI monkeys to develop and improve enzyme replace therapy (ERT) or gene therapy. To establish the system, pedigree analyses and mutation analyses were executed for the colony. Moreover, to develop a new ERT which is effective to symptoms of MPSI on the brain or the heart, ERT for a homozygous monkey with several sugar chain-modified enzymes were prepared.

Academic Significance and Societal Importance of the Research Achievements

指定難病であるライソゾーム病の一種であるムコ多糖症I型の霊長類モデルを世界で初めて発見した。現在行われている治療法は非常に高額であるうえに、脳や心臓の症状に対しては治療効果が不十分である。これらの問題点を改善するためには、新規治療法開発に用いることができる霊長類モデルが必要である。この霊長類モデルを効率的に生産するための基礎データの取得に成功した。また、新規治療法研究に着手した。

Report

(4 results)
  • 2020 Annual Research Report   Final Research Report ( PDF )
  • 2019 Research-status Report
  • 2018 Research-status Report
  • Research Products

    (4 results)

All 2020 2019 2018

All Journal Article (2 results) (of which Peer Reviewed: 1 results) Presentation (2 results) (of which Int'l Joint Research: 1 results,  Invited: 1 results)

  • [Journal Article] 総説 脳におけるヒトと他の霊長類の違い2019

    • Author(s)
      大石高生
    • Journal Title

      BRAIN and NERVE

      Volume: 71 Issue: 7 Pages: 807-813

    • DOI

      10.11477/mf.1416201354

    • ISSN
      1344-8129, 1881-6096
    • Year and Date
      2019-07-01
    • Related Report
      2019 Research-status Report
  • [Journal Article] Neuronal and microglial localization of secreted phosphoprotein 1 (osteopontin) in intact and damaged motor cortex of macaques2019

    • Author(s)
      Sugiyama Yoko、Oishi Takao、Yamashita Akiko、Murata Yumi、Yamamoto Tatsuya、Takashima Ichiro、Isa Tadashi、Higo Noriyuki
    • Journal Title

      Brain Research

      Volume: 1714 Pages: 52-64

    • DOI

      10.1016/j.brainres.2019.02.021

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Presentation] 霊長類研究所のニホンザル繁殖集団若桜群における産子数の解析2020

    • Author(s)
      大石高生,香田啓貴,森本真弓,井戸みゆき,安江美雪,田中洋之
    • Organizer
      第36回日本霊長類学会大会
    • Related Report
      2020 Annual Research Report
  • [Presentation] Two genetic diseases found in the Primate Research Institute2018

    • Author(s)
      Takao Oishi
    • Organizer
      NPRCT-CU SYMPOSIUM “Non-human Primates in Biomedical Research: Industry-Academia Partnerships in Solving Global Health Problems”
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research / Invited

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Published: 2018-04-23   Modified: 2022-01-27  

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