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

Investigation of hematopoietic stem cell transplantation for epidermolysis bullosa

Research Project

Project/Area Number 24249062
Research Category

Grant-in-Aid for Scientific Research (A)

Allocation TypeSingle-year Grants
Section一般
Research Field Dermatology
Research InstitutionHokkaido University

Principal Investigator

SHIMIZU Hiroshi  北海道大学, 医学(系)研究科(研究院), 教授 (00146672)

Co-Investigator(Kenkyū-buntansha) ABE Riichiro  北海道大学, 大学院医学研究科, 准教授 (60344511)
NISHIE Wataru  北海道大学, 北海道大学病院, 講師 (20443955)
FUJITA Yasuyuki  北海道大学, 北海道大学病院, 助教 (80374437)
AKIYAMA Masashi  名古屋大学, 大学院医学系研究科, 教授 (60222551)
KOJIMA Seiji  名古屋大学, 大学院医学系研究科, 教授 (20313992)
Co-Investigator(Renkei-kenkyūsha) NAKAMURA Hideki  北海道大学, 大学院医学研究科, 助手 (60435956)
Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥45,630,000 (Direct Cost: ¥35,100,000、Indirect Cost: ¥10,530,000)
Fiscal Year 2014: ¥7,800,000 (Direct Cost: ¥6,000,000、Indirect Cost: ¥1,800,000)
Fiscal Year 2013: ¥11,960,000 (Direct Cost: ¥9,200,000、Indirect Cost: ¥2,760,000)
Fiscal Year 2012: ¥25,870,000 (Direct Cost: ¥19,900,000、Indirect Cost: ¥5,970,000)
Keywords表皮水疱症 / 幹細胞移植 / 細胞療法 / 造血幹細胞移植 / iPS細胞 / 17型コラーゲン
Outline of Final Research Achievements

Epidermolysis bullosa (EB) is a group of genodermatoses that cause blister formations from the congenital abnormality of anchor proteins between the epidermis and the dermis. There have been several strategies for the treatment of EB, and so far, cell therapies are the most promising approach because of the potential of systemic effects. We have proved that stem cell therapies, including bone marrow transplantation, hematopoietic stem cell transplantation, can ameliorate the phenotype and survival prognosis in the junctional EB model mice that lack type XVII collagen (Col17). In this study we explore more efficient approaches of stem cell therapies for EB, including intramedullary transplantation, mesenchymal stromal/stem cell infusion, and investigate factors in association with transdifferentiation from bone marrow-derived stem cells into keratinocytes.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Annual Research Report
  • 2012 Annual Research Report
  • Research Products

    (15 results)

All 2015 2014 2013 2012

All Journal Article (8 results) (of which Peer Reviewed: 8 results,  Open Access: 1 results,  Acknowledgement Compliant: 1 results) Presentation (7 results) (of which Invited: 4 results)

  • [Journal Article] Skipped exon in COL7A1 determines the clinical phenotypes of dystrophic epidermolysis bullosa.2015

    • Author(s)
      Toyonaga E, Nishie W, Komine M, Murata S, Shinkuma S, Natsuga K, Nakamura H, Ohtsuki M, Shimizu H.
    • Journal Title

      British Journal of Dermatology

      Volume: 172 Issue: 4 Pages: 1141-1144

    • DOI

      10.1111/bjd.13386

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] An annexin A1-FPR1 interaction contributes to necroptosis of keratinocytes in severe cutaneous adverse drug reactions.2014

    • Author(s)
      Saito N, Qiao H, Yanagi T, Shinkuma S, Nishimura K, Suto A, Fujita Y, Suzuki S, Nomura T, Nakamura H, Nagao K, Obuse C, Shimizu H, Abe R.
    • Journal Title

      Science Translational Medicine

      Volume: 6 Issue: 245 Pages: 245-295

    • DOI

      10.1126/scitranslmed.3008227

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Long-term follow-up of cultured epidermal autograft in a patient with recessive dystrophic epidermolysis bullosa2014

    • Author(s)
      Shinkuma S, Sawamura D, Fujita Y, Kawasaki H, Nakamura H, Inoie M, Nishie W, Shimizu H
    • Journal Title

      Acta Derm Venereol

      Volume: 94(1) Issue: 1 Pages: 98-99

    • DOI

      10.2340/00015555-1592

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Stevens-Johnson syndrome/toxic epidermal necrolysis mouse model generated by using PBMCs and the skin of patients.2013

    • Author(s)
      Saito N, Yoshioka N, Abe R, Qiao H, Fujita Y, Hoshina D, Suto A, Kase S, Kitaichi N, Ozaki M, Shimizu H.
    • Journal Title

      Journal of Allergy and Clinical Immunology

      Volume: 131 Issue: 2 Pages: 434-441

    • DOI

      10.1016/j.jaci.2012.09.014

    • Related Report
      2013 Annual Research Report 2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Establishment of a novel experimental model of human angiosarcoma and a VEGF-targeting therapeutic experiment.2013

    • Author(s)
      Hoshina D, Abe R, Yoshioka N, Saito N, Hata H, Fujita Y, Aoyagi S, Shimizu H.
    • Journal Title

      Journal of Dermatological Science

      Volume: 70 Issue: 2 Pages: 116-122

    • DOI

      10.1016/j.jdermsci.2013.02.008

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Jacyk、Ken Natsuga、Hideyuki Ujiie、Hideki Nakamura、Masashi Akiyama、Hiroshi Shimizu2013

    • Author(s)
      Satoru Shinkuma、Wataru Nishie、 Witold K
    • Journal Title

      A Novel Keratin 5 Mutation in an African Family with Epidermolysis Bullosa Simplex Indicates the Importance of the Amino Acid Located at the Boundary Site Between the H1 and Coil 1A Domains

      Volume: 印刷中 Issue: 5 Pages: 585-587

    • DOI

      10.2340/00015555-1538

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Conversion from human haematopoietic stem cells to keratinocytes requires keratinocyte secretory factors2012

    • Author(s)
      Fujita Y, Inokuma D, et al.
    • Journal Title

      Clinical and Experimental Dermatology

      Volume: (印刷中) Issue: 6 Pages: 658-664

    • DOI

      10.1111/j.1365-2230.2011.04312.x

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Type VII collagen deficiency causesdefective tooth enamel formation due to poor differentiation of ameloblasts2012

    • Author(s)
      Umemoto H, Akiyama M, Domon T, Nomura T, Shinkuma S, Ito K, Asaka T, Sawamura D, Uitto J, Uo M, Kitagawa Y, Shimizu H
    • Journal Title

      Am J Pathol

      Volume: 181 Issue: 5 Pages: 1659-71

    • DOI

      10.1016/j.ajpath.2012.07.018

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Presentation] RNA recognition motif of LEMD3 as a key player in the pathogenesis of Buschke-Ollendorff syndrome.2015

    • Author(s)
      Takashima S, Fujita Y, Suzuki S, Saito N, Shinkuma S, Nomura T, Shimizu H.
    • Organizer
      The 71st Annual Meeting of the Society for Investigative Dermatology
    • Place of Presentation
      Atlanta, US
    • Year and Date
      2015-05-06 – 2015-05-09
    • Related Report
      2014 Annual Research Report
  • [Presentation] Highly prevalent SERPINB7 founder mutation causes pseudodominant inheritance pattern in Nagashima-type palmoplantar keratosis.2014

    • Author(s)
      Mizuno O, Nomura T, Suzuki S, Takeda M, Ohguchi Y, Fujita Y, Nishie W, Sugiura K, Akiyama M, Shimizu H
    • Organizer
      The 44th European Society for Dermatological Research (ESDR) Annual Meeting
    • Place of Presentation
      Copenhagen, Denmark
    • Year and Date
      2014-09-10 – 2014-09-13
    • Related Report
      2014 Annual Research Report
  • [Presentation] Epidermolysis bullosa in Japan: from bench to bedside.2014

    • Author(s)
      Fujita Y
    • Organizer
      The 37th Kung-Sun Oh Memorial Lecture
    • Place of Presentation
      Yonsei University (Seoul, Korea)
    • Year and Date
      2014-08-30
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Novel strategies for the treatment of epidermolysis bullosa.2013

    • Author(s)
      Fujita Y
    • Organizer
      The 1st Beijing International Forum of Pediatric Development.
    • Place of Presentation
      Beijing International Conference Center (Beijing, China)
    • Related Report
      2013 Annual Research Report
    • Invited
  • [Presentation] 表皮水疱症の病態と治療2012

    • Author(s)
      藤田靖幸
    • Organizer
      第76回日本皮膚科学会東部支部学術大会
    • Place of Presentation
      ロイトン札幌(北海道)
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Circulating stem cells and wounded skin in epidermolysis bullosa; possible role of chemokines in the recruitment of cells from bone marrow to skin.2012

    • Author(s)
      Yasuyuki Fujita
    • Organizer
      The 37th Annual Meeting of the Japanese Society for Investigative Dermatology
    • Place of Presentation
      ロワジールホテル那覇(沖縄県)
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] 自家培養表皮移植を行い長期観察した 劣性栄養障害型表皮水疱症の1例2012

    • Author(s)
      藤田靖幸
    • Organizer
      第42回日本創傷治癒学会
    • Place of Presentation
      かでる2・7(北海道)
    • Related Report
      2012 Annual Research Report

URL: 

Published: 2013-05-15   Modified: 2019-07-29  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi