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Analyses of hemidesmosomal assembly and disassembly using cultured model cells of inherited blistering skin diseases.

Research Project

Project/Area Number 17K10207
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Dermatology
Research InstitutionNagoya University

Principal Investigator

Hirako Yoshiaki  名古屋大学, 理学研究科, 講師 (50377909)

Project Period (FY) 2017-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2018: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2017: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords表皮 / ヘミデスモソーム / コラーゲン / ラミニン / 細胞-基質間接着 / 表皮-真皮間結合 / 表皮水疱症
Outline of Final Research Achievements

By genome editing, we established epidermis-derived cultured cell lines lacking each of the four major hemidesmosome constituents. Hemidesmosomes were rarely formed in cells lacking plectin or integrin β4 subunit. In type XVII collagen-deficient cells, type II hemidesmosome-like structures were observed, but type I hemidesmosomes were no longer formed. On the other hand, BP230-deficient cells formed type I hemidesmosome-like adhesions. It was suggested that the homotrimer-forming ability of type XVII collagen is important for the assembly of hemidesmosomal constituents into the type I structure.

Academic Significance and Societal Importance of the Research Achievements

ヘミデスモソーム研究には、これまで先天性表皮水疱症患者由来の細胞が用いられ、その発展に大きく寄与してきたが、利用には制限があり、かつ入手も簡単ではなかった。本研究では、細胞バンクから購入した培養株細胞からモデル細胞を作製した。そのため、利用には制限がなく、すでに数十年の研究実績がある株細胞であることから、既存の研究手法による操作も容易である。また、近い将来においては、必要とする研究者に細胞を頒布する予定であり、ヘミデスモソーム研究だけでなく、先天性表皮水疱症の病理解明のための研究にも大いに貢献するものと考えている。

Report

(5 results)
  • 2020 Annual Research Report   Final Research Report ( PDF )
  • 2019 Research-status Report
  • 2018 Research-status Report
  • 2017 Research-status Report
  • Research Products

    (10 results)

All 2020 2019 2018 2017 Other

All Journal Article (3 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 3 results,  Open Access: 1 results) Presentation (6 results) (of which Int'l Joint Research: 1 results) Remarks (1 results)

  • [Journal Article] Unique mouse monoclonal antibodies reactive with maturation-related epitopes on type VII collagen2017

    • Author(s)
      Hayakawa Taihei, Hirako Yoshiaki, et al
    • Journal Title

      EXPERIMENTAL DERMATOLOGY

      Volume: 26 Issue: 9 Pages: 811-819

    • DOI

      10.1111/exd.13306

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Loss of interaction between plectin and type XVII collagen results in epidermolysis bullosa simplex2017

    • Author(s)
      Natsuga K, Nishie W, Nishimura M, Shinkuma S, Watanabe M, Izumi K, Nakamura H, Hirako Y, Shimizu H
    • Journal Title

      Hum Mutat

      Volume: 38 Issue: 12 Pages: 1666-1670

    • DOI

      10.1002/humu.23344

    • NAID

      120006540047

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] C-Terminal Processing of Collagen XVII Induces Neoepitopes for Linear IgA Dermatosis Autoantibodies2017

    • Author(s)
      Toyonaga Ellen、Nishie Wataru、Izumi Kentaro、Natsuga Ken、Ujiie Hideyuki、Iwata Hiroaki、Yamagami Jun、Hirako Yoshiaki、Sawamura Daisuke、Fujimoto Wataru、Shimizu Hiroshi
    • Journal Title

      Journal of Investigative Dermatology

      Volume: 137 Issue: 12 Pages: 2552-2559

    • DOI

      10.1016/j.jid.2017.07.831

    • NAID

      120006540048

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] 基底膜タンパク質ラミニン332の沈着パターン形成におけるプレクチンの機能の検証2020

    • Author(s)
      浅倉亮佑、平子善章
    • Organizer
      第52回日本結合組織学会学術大会
    • Related Report
      2020 Annual Research Report
  • [Presentation] XVII型コラーゲン欠失培養ラット表皮角化細胞株の作製とヘミデスモソーム形成への影響の解析2020

    • Author(s)
      藤岡光、平子善章
    • Organizer
      第52回日本結合組織学会学術大会
    • Related Report
      2020 Annual Research Report
  • [Presentation] Generation of cultured human cells deficient in dermal-epidermal adhesion apparatus by genome editing2019

    • Author(s)
      Yoshiaki Hirako, Ryotaro Kamo and Tatsuya Hiraki
    • Organizer
      Japan - Singapore International Skin Conference 2019
    • Related Report
      2019 Research-status Report
    • Int'l Joint Research
  • [Presentation] XVII型コラーゲンの三量体形成能はヘミデスモソームを介したラミニン沈着において重要な意味をもつ2018

    • Author(s)
      平木辰弥、平子善章
    • Organizer
      第50回日本結合組織学会
    • Related Report
      2018 Research-status Report
  • [Presentation] ヘミデスモソーム構造における2種類のプラーキンタンパク質の役割の解析2017

    • Author(s)
      近藤擁, 加茂遼太郎, 平子善章
    • Organizer
      第49回日本結合組織学会学術大会
    • Related Report
      2017 Research-status Report
  • [Presentation] Distinct roles of two plakin proteins in type I hemidesmosomes2017

    • Author(s)
      近藤擁, 平子善章
    • Organizer
      日本研究皮膚科学会第42回年次学術大会
    • Related Report
      2017 Research-status Report
  • [Remarks] 名古屋大学大学院生命理学専攻細胞生物学グループ

    • URL

      http://www.bio.nagoya-u.ac.jp/laboratory/images/cb.jpg

    • Related Report
      2018 Research-status Report

URL: 

Published: 2017-04-28   Modified: 2025-01-30  

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