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Serine proteinase dysfunction in keratinocyte of Papillon-Lefevre syndrome

Research Project

Project/Area Number 22791048
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeSingle-year Grants
Research Field Dermatology
Research InstitutionHirosaki University

Principal Investigator

TAKIYOSHI Noriko  弘前大学, 医学部・附属病院, 助手 (30568895)

Project Period (FY) 2010 – 2011
Project Status Completed (Fiscal Year 2011)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2011: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2010: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
Keywords過角化 / 遺伝子変異 / 癌 / 表皮細胞 / メラノサイト / 角化 / 遺伝性疾患 / セリンプロテアーゼ / 掌蹠 / セリンプレテアーゼ
Research Abstract

Papillon-Lefevre syndrome (PLS) is characterized by palmoplantar keratosis (PPK) and early-onset periodontitis, caused by deficiency of cathepsin C (CTSC) that activates many serine proteinases. The pathogenesis of PPK in PLS has not been determined. We confirmed that CTSC activates Kallikrein 8 (KLK8) in immortal human keratinocyte line ; HaCaT cell, by stimulating with recombinant CTSC. KLK8 is one of serine proteinase, and has been reported that plays a role in shedding the corneocytes. Furthermore, histopathological finding showed that topical TPA application caused significant hyperkeratosis in the epidermis of CTSC knockout mice, compared to wild type. These findings suggest that an absence of CTSC causes a serine proteinase dysfunction, leads to hyperkeratosis in the epidermi.

Report

(3 results)
  • 2011 Annual Research Report   Final Research Report ( PDF )
  • 2010 Annual Research Report
  • Research Products

    (8 results)

All 2012 2011 2010

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

  • [Journal Article] Buschke-Ollendorff syndrome associated with hypertrophic scar formation : a possible role for LEMD3 mutation2012

    • Author(s)
      Korekawa A, Nakano H, Toyomaki Y, Takiyoshi N, Rokunohe D, Akasaka E, Nakajima K, Sawamura D
    • Journal Title

      Br J Dermatol

      Volume: 166 Issue: 4 Pages: 900-903

    • DOI

      10.1111/j.1365-2133.2011.10691.x

    • Related Report
      2011 Annual Research Report 2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Epidermolysis bullosa pruriginosa with marked phenotypic heterogeneity caused by a recurrent glycine substitution : incomplete penetrance or a latent case?2011

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

      J Dermatol

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Diffuse and focal palmoplantar keratoderma can be caused by a keratin 6c mutation2011

    • Author(s)
      Akasaka E, Nakano H, Nakano A, Toyomaki Y, Takiyoshi N, Rokunohe D, Nishikawa Y, Korekawa A, Matsuzaki Y, Mitsuhashi Y, Sawamura D
    • Journal Title

      Br J Dermatol

      Volume: 165(6) Issue: 6 Pages: 1290-1292

    • DOI

      10.1111/j.1365-2133.2011.10552.x

    • Related Report
      2011 Annual Research Report 2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Epidermolysis bullosa pruriginosa with marked phenotypic heterogeneity caused by a recurrent glycine substitution : incomplete penetrance or a latent case?2011

    • Author(s)
      Takiyoshi N, Nakano H, et al.
    • Journal Title

      The Journal of Dermatology

      Volume: (Epub) Issue: 8 Pages: 718-719

    • DOI

      10.1111/j.1346-8138.2011.01392.x

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Presentation] Functional analyses in Papillon-Lefevre syndrome2011

    • Author(s)
      滝吉典子
    • Organizer
      日本研究皮膚科学会第36回年次学術大会
    • Place of Presentation
      国立京都国際会館
    • Year and Date
      2011-12-10
    • Related Report
      2011 Annual Research Report 2011 Final Research Report
  • [Presentation] Mutational analyses in Papillon-Lefevre syndrome2011

    • Author(s)
      Takiyoshi Noriko
    • Organizer
      The 41st Annual Meeting of the European Society for Dermatological Research (ESDR)
    • Place of Presentation
      バルセロナ(スペイン)
    • Year and Date
      2011-09-09
    • Related Report
      2011 Annual Research Report 2011 Final Research Report
  • [Presentation] カテプシンC遺伝子変異が同定されたPapillon-Lefevre症候群の2家系2011

    • Author(s)
      滝吉典子
    • Organizer
      第26回角化症研究会
    • Place of Presentation
      東京商工会議所
    • Year and Date
      2011-07-30
    • Related Report
      2011 Annual Research Report 2011 Final Research Report
  • [Presentation] カテプシンC遺伝子変異が同定されたPapillon-Lefevre症候群の1家系の報告及び、ケラチノサイト由来細胞におけるカテプシンC発現の検討2010

    • Author(s)
      滝吉典子
    • Organizer
      第17回分子皮膚科学フォーラム
    • Place of Presentation
      ホテルオークラ福岡(福岡市)
    • Year and Date
      2010-07-10
    • Related Report
      2011 Final Research Report 2010 Annual Research Report

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Published: 2010-08-23   Modified: 2016-04-21  

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