T-regulatory cells in primary immune deficiencies.
T-regulatory cells in primary immune deficiencies.
复制标题
原发性免疫缺陷中的T调节细胞。
DOI:
10.1097/aci.0b013e32834cb8fa
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发表时间:
2011-12
影响因子:
2.8
通讯作者:
Chatila TA
中科院分区:
文献类型:
--
作者:
Verbsky JW;Chatila TA
To summarize studies on the development and function of T regulatory cells in primary immune deficiencies. Primary immune deficiencies are associated with high rates of autoimmunity. T regulatory (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. Gene defects underlying PIDs may also compromise the TR cell, leading to breakdown of peripheral tolerance.