原发性免疫缺陷中的调节性 T 细胞。
T-regulatory cells in primary immune deficiencies.
机构信息
Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
出版信息
Curr Opin Allergy Clin Immunol. 2011 Dec;11(6):539-44. doi: 10.1097/ACI.0b013e32834cb8fa.
Abstract
PURPOSE OF REVIEW
To summarize studies on the development and function of T-regulatory (TR) cells in primary immune deficiencies (PIDs).
RECENT FINDINGS
PIDs are associated with high rates of autoimmunity. TR cells, which are critical to the control of autoimmunity, appear involved in the pathogenesis of PID-related autoimmunity. A number of PIDs, including Omenn's syndrome and Wiskott-Aldrich syndrome, have been associated with impaired production and/or function of thymus-derived (natural) TR cells. Recently defined primary immunodeficiencies, including Stim1 deficiency, IL-10 receptor deficiency, and xIAP deficiency, have been associated with defects in TR cells. De-novo generated TR cells from peripheral CD4 conventional T cells is impaired in the hyper IgE syndrome.
SUMMARY
Gene defects underlying PIDs may also compromise the TR cell, leading to breakdown of peripheral tolerance.
摘要
目的综述
总结调节性 T 细胞（TR 细胞）在原发性免疫缺陷病（PID）中的发育和功能的研究进展。
最近发现
PID 与自身免疫的高发病率相关。TR 细胞对自身免疫的控制至关重要，似乎参与了 PID 相关自身免疫的发病机制。许多 PID，包括 Omenn 综合征和 Wiskott-Aldrich 综合征，与胸腺来源（天然）TR 细胞的产生和/或功能受损有关。最近定义的原发性免疫缺陷病，包括 Stim1 缺乏、IL-10 受体缺乏和 xIAP 缺乏，与 TR 细胞缺陷有关。在高 IgE 综合征中，外周 CD4 常规 T 细胞中产生的新生成的 TR 细胞受损。
总结
PID 相关的基因缺陷也可能破坏 TR 细胞，导致外周耐受的破坏。
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