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Review
. 2011 Dec;11(6):539-44.
doi: 10.1097/ACI.0b013e32834cb8fa.

T-regulatory cells in primary immune deficiencies

James W Verbsky  1 , Talal A Chatila
Affiliations
Review

T-regulatory cells in primary immune deficiencies

James W Verbsky et al. Curr Opin Allergy Clin Immunol. 2011 Dec.

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.

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Conflict of interest statement

Conflict of interest:

The authors acknowledge grant support by the National Institutes of Health to T.A.C. (5R01AI085090 and 5R01AI073731) and J.W.V. (1K08AI072023)

References

    1. Sakaguchi S. Naturally arising Foxp3-expressing CD25+CD4+ regulatory T cells in immunological tolerance to self and non-self. Nat Immunol. 2005;6:345–352. - PubMed
    1. Fontenot JD, Rudensky AY. A well adapted regulatory contrivance: regulatory T cell development and the forkhead family transcription factor Foxp3. Nat Immunol. 2005;6:331–337. - PubMed
    1. Chatila TA. Role of regulatory T cells in human diseases. Journal of Allergy and Clinical Immunology. 2005;116:949–959. - PubMed
    1. Fontenot JD, Gavin MA, Rudensky AY. Foxp3 programs the development and function of CD4+CD25+ regulatory T cells.[comment] Nature Immunology. 2003;4:330–336. - PubMed
    1. Hori S, Nomura T, Sakaguchi S. Control of regulatory T cell development by the transcription factor Foxp3.[comment] Science. 2003;299:1057–1061. - PubMed

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