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

Factors inhibit serine protease activity and echance epidermal differentiation in Papillon-Lefevre syndrome

Research Project

Project/Area Number 24591621
Research Category

Grant-in-Aid for Scientific Research (C)

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

Principal Investigator

TAKIYOSHI Noriko  弘前大学, 医学部附属病院, 助教 (30568895)

Co-Investigator(Kenkyū-buntansha) NAKANO Hajime  弘前大学, 大学院医学研究科, 准教授 (90281922)
Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywordscathepsin C / inflamation / hyperkeratosis / acanthosis / カテプシンC / パピヨン・ルフェーブル症候群 / セリンプロテアーゼ / 過角化 / 外的刺激
Outline of Final Research Achievements

Total loss of Cathepsin C ( CTSC ) activity cause Papillon-Lefevre syndrome ( PLS ), which characterized by palmoplantar keratoderma ( PPK ). CTSC knock out mice naturaly lack PPK, so we examined whether inflammation trigger a change of epidermal differentitation in CTSC knock out mice. We made four groups, knock out mice and wild type mice with or without application of TPA. We confirmed that knock out mice with application of TPA had feeature of palmplanterkeratosis,remarkable hyperkeratosis and acanthosis histologically.
This research sugesut that skin manifestation of Pappilion Lefevre syndrome caused by irritation,so skin change localize palmplanter,knee,elbow in PLS.

Report

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

    (10 results)

All 2014 2013 2012 Other

All Journal Article (4 results) (of which Peer Reviewed: 3 results) Presentation (6 results)

  • [Journal Article] 点状掌蹠角化症の2家系例2014

    • Author(s)
      赤坂英二郎、中野 創、滝吉典子、澤村大輔
    • Journal Title

      第28回角化症研究会記録集

      Volume: 28 Pages: 78-81

    • Related Report
      2013 Research-status Report
  • [Journal Article] Papillon-Lefèvre syndrome: Impact of cathepsin C activation on the phenotypic expression.2013

    • Author(s)
      Takiyoshi N, Nakano H, Sawamura D
    • Journal Title

      Hirosaki Med J

      Volume: 64 Pages: 65-70

    • NAID

      110009614131

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 遺伝性角化症のスキルアップ「分類と診断のポイント,最近の話題」2013

    • Author(s)
      中野 創
    • Journal Title

      MB Derma

      Volume: 203 Pages: 73-78

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Multiple aplasia cutis congenita lesions located along Blaschko's lines in a patient with tetralogy of Fallot-A.2012

    • Author(s)
      Rokunohe D
    • Journal Title

      J Dermatol Case Rep

      Volume: 6 Issue: 2 Pages: 40-42

    • DOI

      10.3315/jdcr.2012.1096

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Presentation] Impact of Cathepsin C activity on keratinaization2014

    • Author(s)
      滝吉 典子
    • Organizer
      表皮細胞研究会
    • Place of Presentation
      鹿児島市
    • Year and Date
      2014-12-06
    • Related Report
      2014 Annual Research Report
  • [Presentation] Papillon-Lefèvre syndrome ; total loss of cathepsin C activity may be required for disease expression.2012

    • Author(s)
      Takiyoshi N
    • Organizer
      42th Annual Meeting of the European Society for Dermatological Research (ESDR 2012)
    • Place of Presentation
      Venice(Italy)
    • Related Report
      2012 Research-status Report
  • [Presentation] Papillon-Lefèvre syndrome; five novel mutation of CTSC and functional analyses of a single nucleotide polymorphism.2012

    • Author(s)
      Takiyoshi N
    • Organizer
      The 37th Annual Meeting of the Japanese Society for Investigative Dermatology
    • Place of Presentation
      Okinawa(沖縄県)
    • Related Report
      2012 Research-status Report
  • [Presentation] カテプシンC遺伝子変異が同定されたPapillon-Lefèvre症候群:本邦4家系の報告2012

    • Author(s)
      滝吉 典子
    • Organizer
      第19回分子皮膚科学フォーラム
    • Place of Presentation
      ホテル青森(青森県)
    • Related Report
      2012 Research-status Report
  • [Presentation] 点状掌蹠角化症の2家系例

    • Author(s)
      赤坂英二郎、中野 創、澤村大輔
    • Organizer
      第28回角化症研究会
    • Place of Presentation
      東京商工会議所
    • Related Report
      2013 Research-status Report
  • [Presentation] Richner-Hanhart症候群;臨床症状と血中チロシン濃度との関係

    • Author(s)
      滝吉 典子
    • Organizer
      第27回角化症研究会
    • Place of Presentation
      東京商工会議所(東京都)
    • Related Report
      2012 Research-status Report

URL: 

Published: 2013-05-31   Modified: 2019-07-29  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi