Elucidating pathomechanism for bullous pemphigoid by molecular imaging of collagen XVII.

2010 Fiscal Year Self-evaluation Report

• Project/Area Number: 20689021
• Research Category: Grant-in-Aid for Young Scientists (A)
• Allocation Type: Single-year Grants
• Research Field: Dermatology
• Research Institution: Hokkaido University
• Principal Investigator: NISHIE Wataru Hokkaido University, 大学病院, 講師 (20443955)
• Project Period (FY): 2008 – 2011
• Keywords: 生体分子 / 細胞・組織 / タンパク質 / 酵素 / 応用動物

Research Abstract

本研究の目的は、自己免疫性水疱症の中で最も患者数の多い水疱性類天疱瘡(bullous pemphigoid ; BP)の病態メカニズムを解明する事である。その手法として、BPの自己抗原であり表皮基底細胞ヘミデスモゾーム構成分子の1つのXVII型コラーゲン(COL17)を分子イメージングするという方法を用いた。本研究により、BP患者自己抗体がCOL17分子へ結合した際のCOL17分子の動態を直接視覚的に捉えることが可能となり、BP患者自己抗体によるCOL17に対する直接的病原性有無の解明のほか、自己抗体により切断されたCOL17断片の生理学的機能などを解明することが可能となる。

Research Products

• [Journal Article] Ectodomain shedding generates neoepitopes on collagen XVII, the major autoantigen for bullous pemphigoid. (2010)
  • Author(s): Nishie W, Lamer S, Schlosser A, Licarete E, Franzke C, Hofmann S, Jackow J, Sitaru C, Bruckner-Tuderman L.
  • Journal Title: J Immunol 185 Pages: 4938-4947
  • Peer Reviewed
• [Journal Article] Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid. (2010)
  • Author(s): atsuga K, Nishie W, Shinkuma S, Moriuchi R, Shibata M, Nishimura M, Hashimoto T, Shimizu H.
  • Journal Title: Br J Dermatol 162 Pages: 513-517
  • Peer Reviewed
• [Journal Article] Complete paternal isodisomy of chromosome 17 in junctional epidermolysis bullosa with pyloric atresia. (2010)
  • Author(s): Natsuga K, Nishie W, Arita K, Shinkuma S, Nakamura H, Kubota S, Imakado S, Akiyama M, Shimizu H
  • Journal Title: J Invest Dermatol 130 Pages: 2671-2674
  • Peer Reviewed
• [Journal Article] A novel humanized neonatal autoimmune blistering skin disease model induced by maternally transferred antibodies. (2009)
  • Author(s): Nishie W, Sawamura D, Natsuga K, Shinkuma S, Goto M, Shibaki A, Ujiie H, Olasz E, Yancey KB, Shimizu H.
  • Journal Title: J Immunol 183 Pages: 4088-4093
  • Peer Reviewed
• [Journal Article] Dynamic Interactions of Epidermal Collagen XVII with the Extracellular Matrix : Laminin 332 as a Major Binding Partner.
  • Author(s): Nishie W, Kiritsi D, Nystrom A, Hofmann S, Bruckner-Tuderman L.
  • Journal Title: Am J Pathol in press.
  • Peer Reviewed
• [Presentation] Dynamics of shed ectodomain of collagen XVII on extracellular matrix (2010)
  • Author(s): Nishie, W, Bruckner-Tuderman L
  • Organizer: Annual Meeting of the German and British Societies for Matrix Biology
  • Place of Presentation: Frankfurt / Main Germany
  • Year and Date: 20100300
• [Presentation] Role of distinctive proteins interacting with collagen XVII in extracellular matrix. (2010)
  • Author(s): Nishie W, Bruckner-Tuderman L.
  • Organizer: The 35th Annual Meeting of the Japanese Society of Invesitigative Dermatology
  • Place of Presentation: Wakayama
  • Year and Date: 2010-12-03
• [Presentation] Novel cleavage sites and specific neoepitopes on shed ectodomain of collagen XVII/BP180: role in autoimmune blistering diseases. (2010)
  • Author(s): Nishie W, Lamer S, Schlosser A, Licarete E, Franzke CW, Hofmann SC, Jackow J, Sitaru C, Bruckner-Tuderman L.
  • Organizer: The 41st Annual Meeting of the European Society for Dermatological Research
  • Place of Presentation: Helsinki, Finland
  • Year and Date: 2010-09-10
• [Presentation] Transmembrane collagen XVII: ectodomain shedding generates neo-epitopes. (2009)
  • Author(s): Nishie W, Has C, Bruckner-Tuderman L.
  • Organizer: Gordon Research Conference, Collagen
  • Place of Presentation: New London, NH, USA
  • Year and Date: 2009-07-20