強皮症と自己免疫 Scleroderma and Autoimmunity

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著者

    • 藤本 学 FUJIMOTO Manabu
    • 国立国際医療センター研究所細胞組織再生医学研究部細胞修飾生体反応研究室 Department of Regenerative Medicine, Research Institute, International Medical Center of Japan

抄録

Scleroderma is an autoimmune disorder characterized by fibrosis in the skin and is divided into two types: systemic sclerosis and localized scleroderma. The etiology and pathogenesis of scleroderma are still unclear. Autoantibodies have been useful diagnostic tools and their clinical significance is reviewed in this article. Furthermore, recent studies have revived the idea that autoantibodies and B lymphocytes play principal roles in systemic autoimmune diseases. In the tight skin (Tsk/+) mouse, a genetic model of systemic sclerosis, B cells exhibit a hyper-responsive phenotype due to the disruption of the CD22/SHP-1 negative regulatory pathway. This inhibitory pathway is regulated by CD19, and the loss of CD19 expression significantly reduces skin fibrosis and autoantibody production in Tsk/+ mice. Two features of scleroderma, fibrosis and autoimmunity, thus affect each other, which may provide a clue to comprehending the pathogenesis of the disease.

収録刊行物

  • 日本皮膚科学会雑誌

    日本皮膚科学会雑誌 112(6), 785-792, 2002-05-20

    Japanese Dermatological Association

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各種コード

  • NII論文ID(NAID)
    10008880592
  • NII書誌ID(NCID)
    AN00196602
  • 本文言語コード
    JPN
  • 資料種別
    REV
  • ISSN
    0021499X
  • NDL 記事登録ID
    025117272
  • NDL 請求記号
    Z19-202
  • データ提供元
    CJP書誌  NDL  J-STAGE 
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