Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comment
. 2006 Jun;116(6):1473-5.
doi: 10.1172/JCI28880.

IPEX and the role of Foxp3 in the development and function of human Tregs

Séverine Le Bras  1 , Raif S Geha
Affiliations
Comment

IPEX and the role of Foxp3 in the development and function of human Tregs

Séverine Le Bras et al. J Clin Invest. 2006 Jun.

Abstract

Genetic defects in the transcription factor forkhead box protein P3 (Foxp3) cause immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX). IPEX is thought to be due to a defect in naturally arising CD4+ Tregs. In this issue of the JCI, Bacchetta and colleagues demonstrate that patients with IPEX and missense mutations in Foxp3 provide insight into the role of various domains of Foxp3 in the development and function of Tregs (see the related article beginning on page 1713).

PubMed Disclaimer

Comment on

References

    1. Dejaco C., Duftner C., Grubeck-Loebenstein B., Schirmer M. Imbalance of regulatory T cells in human autoimmune diseases. Immunology. 2006;117:289–300. - PMC - PubMed
    1. Sakaguchi S., Sakaguchi N., Asano M., Itoh M., Toda M. Immunologic self-tolerance maintained by activated T cells expressing IL-2 receptor alpha-chains (CD25). Breakdown of a single mechanism of self-tolerance causes various autoimmune diseases. J. Immunol. 1995;155:1151–1164. - PubMed
    1. Sullivan K.E., McDonald-McGinn D., Zackai E.H. CD4(+) CD25(+) T-cell production in healthy humans and in patients with thymic hypoplasia. Clin. Diagn. Lab. Immunol. 2002;9:1129–1131. - PMC - PubMed
    1. Bensinger S.J., Bandeira A., Jordan M.S., Caton A.J., Laufer T.M. Major histocompatibility complex class II-positive cortical epithelium mediates the selection of CD4(+)25(+) immunoregulatory T cells. J. Exp. Med. 2001;194:427–438. - PMC - PubMed
    1. Fontenot J.D., Rudensky A.Y. A well adapted regulatory contrivance: regulatory T cell development and the forkhead family transcription factor Foxp3. Nat. Immunol. 2005;6:331–337. - PubMed

MeSH terms

Substances