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Development of the deaminoneuraminic acid therapy for treatment of congenital disorders of glycosylation Ib

Research Project

Project/Area Number 15K14918
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Applied molecular and cellular biology
Research InstitutionNagoya University

Principal Investigator

Kitajima Ken  名古屋大学, 生物機能開発利用研究センター, 教授 (80192558)

Project Period (FY) 2015-04-01 – 2017-03-31
Project Status Completed (Fiscal Year 2016)
Budget Amount *help
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2016: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2015: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Keywordsデアミノノイラミン酸 / 先天性 / マンノースセラピー / 代謝 / 糖質 / 治療 / シアル酸 / 代謝酵素 / 先天性糖鎖合成異常症
Outline of Final Research Achievements

The objective of this study is to development of the KDN therapy as a new drug for CDG1b patients. In this disorder, glycan amount is reduced due to a deficiency of Man. The reduced glycans are rescued by the mannose therapy; however, the development of new method would be desired, if possible. In this study, we thus try to demonstrate the effectiveness of the new method. As a result, we attained the establishment of analytical methods for metabolic products and the amount of cell surface glycan chains. On the other hand, CDG1b model cell lines could not be established within the research period, even after many efforts. Notably, we succeeded in demonstrating that KDN is incorporated into cells through ponocytosis as well as through one of monocarboxylate transporters unlike a major sialic acid species Neu5Ac.

Report

(3 results)
  • 2016 Annual Research Report   Final Research Report ( PDF )
  • 2015 Research-status Report
  • Research Products

    (3 results)

All 2016 2015

All Presentation (3 results)

  • [Presentation] Uptake mechanism of deaminoneuraminic acid in mammalian cells.2016

    • Author(s)
      岩木佑弥、佐藤ちひろ、北島健
    • Organizer
      第13回若手の力フォーラム
    • Place of Presentation
      岐阜大学応用生物科学部
    • Year and Date
      2016-11-26
    • Related Report
      2016 Annual Research Report
  • [Presentation] 哺乳類細胞におけるデアミノノイラミン酸の取込み機構の解析2016

    • Author(s)
      岩木佑弥、郷慎司、佐藤ちひろ、北島健
    • Organizer
      第35回日本糖質学会年会2016年9月1-3日
    • Place of Presentation
      高知市文化プラザ
    • Year and Date
      2016-09-01
    • Related Report
      2016 Annual Research Report
  • [Presentation] 液体窒素を用いた急速凍結細胞回収法によるシアル酸代謝物定量の改良2015

    • Author(s)
      岩木佑弥, 宮田真路, 佐藤ちひろ, 北島 健
    • Organizer
      第34回日本糖質学会年会
    • Place of Presentation
      東京大学安田講堂
    • Year and Date
      2015-07-31
    • Related Report
      2015 Research-status Report

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Published: 2015-04-16   Modified: 2019-12-27  

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