• Search Research Projects
  • Search Researchers
  • How to Use
  1. Back to previous page

Investigation of the pathogenesis and diagnostic method of age-related EFEMP1 amyloidosis

Research Project

Project/Area Number 17K08987
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Laboratory medicine
Research InstitutionKumamoto University

Principal Investigator

Tasaki Masayoshi  熊本大学, 大学院生命科学研究部(保), 助教 (50613402)

Co-Investigator(Kenkyū-buntansha) 安東 由喜雄  熊本大学, 大学院生命科学研究部(医), 教授 (20253742)
大林 光念  熊本大学, 大学院生命科学研究部(保), 教授 (90361899)
山下 太郎  熊本大学, 病院, 非常勤診療医師 (90381003)
Project Period (FY) 2017-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2020: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2018: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2017: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Keywordsアミロイドーシス / アミロイド / 老化 / EFEMP1 / 静脈 / 質量分析
Outline of Final Research Achievements

Amyloidosis is a group of disorders characterized by the deposition of amyloid fibrils in extracellular space. We newly identified EGF-containing fibulin-like extracellular matrix protein 1(EFEMP1)as a novel amyloid precursor protein. However, the pathogenesis is not fully understood. In this study, we investigated the pathogenesis of novel EFEMP1 amyloidosis. Our study revealed the following findings. 1) The occurrence and amount of EFEMP1 amyloid increased with age in GI tract, lung and bladder. 2) C-terminal region of EFEMP1 formed nonbranching fibrils. 3) Many proteins were identified from amyloid-laden tissues by mass spectrometry. 4) FSB staining and immunohistochemistry with anti EFEMP1 antibodies are superior to detect EFEMP1 amyloid deposits in tissues.

Academic Significance and Societal Importance of the Research Achievements

本研究では、新規アミロイド原因タンパク質としてEFEMP1を同定し、本アミロイドーシスの病態の一端を明らかにした。これまで、長年見過ごされていた病態を解明できたことで、本研究の成果は、今後の治療法や検査法の開発に繋がると考えられる。また、本研究成果が評価され、EFEMP1は新型のアミロイド原因タンパク質として、国際アミロイドーシス学会のアミロイド分類委員会より承認を得た(*Benson et al., Amyloid 2020)。アミロイドーシス研究において、新たなアミロイド原因タンパク質を見出したことは極めて重要な成果であり、アミロイド形成メカニズムを明らかにするのに役立つことが期待される。

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

    (20 results)

All 2021 2020 2019 2018 2017 Other

All Int'l Joint Research (3 results) Journal Article (10 results) (of which Int'l Joint Research: 3 results,  Peer Reviewed: 10 results,  Open Access: 4 results) Presentation (6 results) (of which Int'l Joint Research: 2 results,  Invited: 1 results) Book (1 results)

  • [Int'l Joint Research] University of Pavia(イタリア)

    • Related Report
      2019 Research-status Report
  • [Int'l Joint Research] University of Pavia(イタリア)

    • Related Report
      2018 Research-status Report
  • [Int'l Joint Research] University of Pavia(イタリア)

    • Related Report
      2017 Research-status Report
  • [Journal Article] EGF-containing fibulin-like extracellular matrix protein 1 amyloid incidentally found in surgically resected specimens of Colon cancer: a case report emphasising on an unrecognised type2021

    • Author(s)
      Tsukamoto Yoshitane、Tasaki Masayoshi、Fujii Hitoshi、Tsujie Masaki、Ueda Mitsuharu
    • Journal Title

      Amyloid

      Volume: 28 Issue: 2 Pages: 136-137

    • DOI

      10.1080/13506129.2020.1841624

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Apolipoprotein AI amyloid deposits in the ligamentum flavum in patients with lumbar spinal canal stenosis2020

    • Author(s)
      Tasaki Masayoshi、Okada Masamitsu、Yanagisawa Akihiro、Nomura Toshiya、Matsushita Hiroaki、Ueda Akihiko、Inoue Yasuteru、Masuda Teruaki、Misumi Yohei、Yamashita Taro、Nakamura Takayuki、Miyamoto Takeshi、Obayashi Konen、Ando Yukio、Ueda Mitsuharu
    • Journal Title

      Amyloid

      Volume: 28 Issue: 2 Pages: 1-6

    • DOI

      10.1080/13506129.2020.1858404

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Simple, reliable detection of amyloid in fat aspirates using the fluorescent dye FSB: prospective study in 206 patients.2019

    • Author(s)
      Tasaki M, Milani P, Foli A, Verga L, Obici L, Basset M, Bozzola M, Ferraro G, Nuvolone M, Morbini P, Capello G, Ueda M, Obayashi K, Paulli M, Ando Y, Merlini G, Palladini G, Lavatelli F.
    • Journal Title

      Blood

      Volume: 134 Issue: 3 Pages: 320-323

    • DOI

      10.1182/blood.2019000420

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] A novel age-related venous amyloidosis derived from EGF-containing fibulin-like extracellular matrix protein 12019

    • Author(s)
      Tasaki M, Ueda M, Hoshii Y, Mizukami M, Matsumoto S, Nakamura M, Yamashita T, Ueda A, Misumi Y, Masuda T, Inoue Y, Torikai T, Nomura T, Tsuda Y, Kanenawa K, Isoguchi A, Okada M, Matsui H, Obayashi K, Ando Y.
    • Journal Title

      The Journal of Pathology

      Volume: 247 Issue: 4 Pages: 444-455

    • DOI

      10.1002/path.5203

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient.2019

    • Author(s)
      Swuec P, Lavatelli F, Tasaki M, Paissoni C, Rognoni P, Maritan M, Brambilla F, Milani P, Mauri P, Camilloni C, Palladini G, Merlini G, Ricagno S, Bolognesi M.
    • Journal Title

      Nature Communiations

      Volume: 10 Issue: 1 Pages: 1269-1269

    • DOI

      10.1038/s41467-019-09133-w

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] ATR-FTIR Spectroscopy Supported by Multivariate Analysis for the Characterization of Adipose Tissue Aspirates from Patients Affected by Systemic Amyloidosis2019

    • Author(s)
      Ami D, Mereghetti P, Foli A, Tasaki M, Milani P, Nuvolone M, Palladini G, Merlini G, Lavatelli F, Natalello A
    • Journal Title

      Analytical Chemistry

      Volume: 91 Issue: 4 Pages: 2894-2900

    • DOI

      10.1021/acs.analchem.8b05008

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Genetic and clinical characteristics of hereditary transthyretin amyloidosis in endemic and non-endemic areas: experience from a single-referral center in Japan.2018

    • Author(s)
      Yamashita T, Ueda M, Misumi Y, Masuda T, Nomura T, Tasaki M, Takamatsu K, Sasada K, Obayashi K, Matsui H, Ando Y.
    • Journal Title

      Journal of Neurology

      Volume: 265 Issue: 1 Pages: 134-140

    • DOI

      10.1007/s00415-017-8640-7

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Sushi repeat-containing protein 1: a novel disease-associated molecule in cerebral amyloid angiopathy.2017

    • Author(s)
      Inoue Y, Ueda M, Tasaki M, Takeshima A, Nagatoshi A, Masuda T, Misumi Y, Kosaka T, Nomura T, Mizukami M, Matsumoto S, Yamashita T, Takahashi H, Kakita A, Ando Y.
    • Journal Title

      Acta Neuropathologica

      Volume: 134 Issue: 4 Pages: 605-617

    • DOI

      10.1007/s00401-017-1720-z

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Serum amyloid P component: A novel potential player in vessel degeneration in CADASIL.2017

    • Author(s)
      Nagatoshi A, Ueda M, Ueda A, Tasaki M, Inoue Y, Ma Y, Masuda T, Mizukami M, Matsumoto S, Kosaka T, Kawano T, Ito T, Ando Y.
    • Journal Title

      Journal of the Neurological Sciences

      Volume: 379 Pages: 69-76

    • DOI

      10.1016/j.jns.2017.05.033

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] A novel transthyretin variant V28S (p.V48S) with a double-nucleotide substitution in the same codon.2017

    • Author(s)
      Okada M, Misumi Y, Ueda M, Yamashita T, Masuda T, Tasaki M, Ando Y.
    • Journal Title

      Amyloid

      Volume: 24 Issue: 4 Pages: 231-232

    • DOI

      10.1080/13506129.2017.1381082

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Presentation] EGF-containing fibulin-like extracellular matrix protein 1: A novel age-related amyloid precursor protein2020

    • Author(s)
      Masayoshi Tasaki, Mitsuharu Ueda, Yoshinobu Hoshii, Aito Isoguchi, Toshiya Nomura, Masamitsu Okada, Yasuteru Inoue, Teruaki Masuda, Yohei Misumi, Taro Yamashita, Konen Obayashi, Yukio Ando
    • Organizer
      The XVII International Symposium On Amyloidosis
    • Related Report
      2020 Annual Research Report
    • Int'l Joint Research
  • [Presentation] EFEMP1アミロイドの沈着頻度および至適病理学的診断法の検討2019

    • Author(s)
      田崎雅義、植田光晴、大林光念、安東由喜雄
    • Organizer
      第66回日本臨床検査医学会学術集会
    • Related Report
      2019 Research-status Report
  • [Presentation] 質量分析装置を用いた新規アミロイド原因蛋白質、EFEMP1の同定2019

    • Author(s)
      田崎雅義、植田光晴、星井嘉信、磯口藍斗、山下太郎、大林光念、安東由喜雄
    • Organizer
      第59回日本臨床化学会学術集会
    • Related Report
      2019 Research-status Report
  • [Presentation] EFEMP1 C末端領域のアミロイド形成能および細胞毒性2019

    • Author(s)
      田崎雅義、礒口藍斗、植田光晴、星井嘉信、岡田匡充、野村隼也、井上泰輝、増田曜章、三隅洋平、山下太郎、大林光念、安東由喜雄
    • Organizer
      第7回日本アミロイドーシス学会学術集会
    • Related Report
      2019 Research-status Report
  • [Presentation] アミロイドーシスの診断法開発と臨床応用2019

    • Author(s)
      田崎雅義
    • Organizer
      第66回日本臨床検査医学会学術集会
    • Related Report
      2019 Research-status Report
    • Invited
  • [Presentation] Characteristics of TTR fragmentation pattern in ATTR patients carrying various mutations2018

    • Author(s)
      Masayoshi Tasaki, Francesca Lavatelli, Angelo M. Minnella, Marco Di Girolamo, Elena Antoniazzi, Margerita Bozzola, Giovanni Ferraro, Mario Nuvolone, Giovanni Palladini, Konen Obayashi, Yukio Ando, Giampaolo Merlini, Laura Obici
    • Organizer
      XVIth International Symposium on Amyloidosis
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Book] 21世紀の疾患: 神経関連アミロイドーシス2020

    • Author(s)
      安東由喜雄、礒口藍斗、一木裕子、井上泰輝、植田光晴、大林光念、岡田匡充、城野博史、田崎雅義、野村隼也、増田曜章、松下博昭、三隅洋平、山下太郎、渡邊礼美
    • Total Pages
      136
    • Publisher
      医学と看護社
    • ISBN
      9784909888075
    • Related Report
      2019 Research-status Report

URL: 

Published: 2017-04-28   Modified: 2022-01-27  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi