IPEX症候群とヒト Treg 細胞  [in Japanese] IPEX syndrome and human Treg cells  [in Japanese]

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  免疫系には免疫抑制機能に特化した制御性T細胞(Treg)とよばれる少数のCD4<sup>+</sup>CD25<sup>+</sup> T細胞サブセットが存在する.この細胞は,自己免疫やアレルギー,炎症といった過剰な免疫反応を抑制して免疫恒常性の維持において非常に重要な役割を果たしている.Tregのマスター遺伝子として<i>FOXP3</i>遺伝子が機能するという発見により,Tregの生理的意義が明確に証明され,その発生・分化と抑制機能の分子メカニズムを解明するうえで重要な進歩がもたらされた.<br>   このTregの欠損や機能低下によって生じる疾患がIPEX (immune dysregulation, polyendocrinopathy, enteropathy, and X-linked)症候群である.この疾患は,I 型糖尿病や甲状腺機能低下症などの多発性内分泌異常,難治性下痢などを主症状とし,さらには自己免疫性と考えられる貧血,血小板減少,腎炎など多彩な症状を呈する.<br>   現在まで報告されているIPEX症候群の臨床像,分子学的異常とヒトTreg細胞の機能に関して概説する.<br>

  CD4<sup>+</sup>CD25<sup>+</sup> T cells which have also been described as regulatory T cells (Treg), have immune inhibitory functions in the immune system. This population inhibits excessive immune responses, such as those present in patients with autoimmune disease, allergy and inflammation, and plays an important role in maintenance of immunological homeostasis. It has been demonstrated that the <i>FOXP3</i> gene is a master gene for a transcriptional factor of Tregs. This finding has led to the elucidation of the Treg functions during development, differentiation and immune suppression.<br>   Either a deficiency or dysfunction of Tregs results in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, and X-linked) syndrome. The clinical features of IPEX syndrome include chronic dermatitis, enteropathy characterized by severe and refractory diarrhea, and autoimmune endocrinopathy, such as early-onset insulin-dependent diabetes mellitus, thyroiditis, or both. This syndrome is also associated with various symptoms such as anemia, thrombocytopenia and nephritis which may be caused by an autoimmune response.<br>   We herein describe the clinical and molecular characteristics of patients with IPEX syndrome and also elucidate the function of human Treg cells.<br>


  • Japanese Journal of Clinical Immunology

    Japanese Journal of Clinical Immunology 33(4), 196-206, 2010-08-31

    The Japan Society for Clinical Immunology

References:  66

Cited by:  1


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