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Role of inherited defects decreasing Fas function in autoimmunity.

机译：遗传缺陷降低Fas功能在自身免疫中的作用。

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摘要

Fas is a death receptor belonging to the TNFR superfamily and induces cell apoptosis by both activating a caspase cascade and altering mitochondria. In the immune system, Fas is involved in the switching-off of the immune responses and cell mediated cytotoxicity. In humans, genetic defects decreasing Fas function cause the Autoimmune Lymphoproliferative Syndrome (ALPS) where autoimmunities are associated with accumulation of polyclonal lymphocytes in the secondary lymphoid tissues and expansion of T cells lacking both CD4 and CD8 (DN cells). Expansion of DN cells is absent in an ALPS variant, named Dianzani's Autoimmune Lymphoproliferative Disease (DALD). The observation that DALD patients' families display increased frequency of autoimmune diseases different from ALPS suggests that defects of Fas function may also play a role in development of "common" autoimmune diseases. This possibility is supported by detection of defective Fas function in substantial proportions of patients with the multiple autoimmune syndrome or aggressive forms of type 1 diabetes or multiple sclerosis. This article reviews data suggesting that development of autoimmune/lymphoproliferative patterns may involve several alterations hitting the Fas system, but might also involve alterations in other systems contributing to the switching-off or proliferation of lymphocytes.
机译：Fas是属于TNFR超家族的死亡受体，并且通过激活半胱天冬酶级联反应和改变线粒体来诱导细胞凋亡。在免疫系统中，Fas参与了免疫应答的关闭和细胞介导的细胞毒性作用。在人类中，降低Fas功能的遗传缺陷会导致自身免疫性淋巴组织增生综合症（ALPS），其中自身免疫性与次级淋巴组织中多克隆淋巴细胞的积累以及缺乏CD4和CD8的T细胞（DN细胞）的扩增有关。称为Dianzani自身免疫性淋巴增生性疾病（DALD）的ALPS变体不存在DN细胞的扩增。 DALD患者家属显示出与ALPS不同的自身免疫性疾病发生频率增加的观察结果表明，Fas功能缺陷也可能在“常见”自身免疫性疾病的发展中起作用。通过检测患有多种自身免疫综合症或1型糖尿病或多发性硬化症的侵袭性形式的患者中相当比例的Fas功能缺陷，可以支持这种可能性。本文对数据进行了分析，这些数据表明自身免疫/淋巴增生模式的发展可能涉及击中Fas系统的几种改变，但也可能涉及其他系统的改变，从而导致淋巴细胞的关闭或增殖。

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