Skip Navigation LinksHome > December 2011 - Volume 11 - Issue 6 > T-regulatory cells in primary immune deficiencies
Current Opinion in Allergy & Clinical Immunology:
doi: 10.1097/ACI.0b013e32834cb8fa
Primary immune deficiency disease: Edited by Amos Etzioni and Ramsey Fuleihan

T-regulatory cells in primary immune deficiencies

Verbsky, James W.a; Chatila, Talal A.b

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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.

© 2011 Lippincott Williams & Wilkins, Inc.

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