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Immunologic Research

Erschienen in:

01.07.2007

IPEX, FOXP3 and regulatory T-cells: a model for autoimmunity

verfasst von: Hans D. Ochs, Eleonora Gambineri, Troy R. Torgerson

Erschienen in: Immunologic Research | Ausgabe 1-3/2007

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Abstract

FOXP3 is the key mediator of regulatory T-cell development in the thymus. Naturally occurring mutations of FOXP3 interfere with this process, resulting in the generation of autoaggressive lymphocyte clones that are directly responsible for the syndrome Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) in humans and scurfy in mice. Stem cell transplantation is the only cure for IPEX patients. The study of this rare disease has provided important insight into the mechanisms of immunosuppression, autoimmunity and tolerance and future studies may lead to novel strategies to treat not only patients with IPEX, but also those suffering from autoimmunity, graft-versus-host disease or cancer.

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Titel: IPEX, FOXP3 and regulatory T-cells: a model for autoimmunity
verfasst von: Hans D. Ochs
Eleonora Gambineri
Troy R. Torgerson
Publikationsdatum: 01.07.2007
Verlag: Humana Press Inc
Erschienen in: Immunologic Research / Ausgabe 1-3/2007
Print ISSN: 0257-277X
Elektronische ISSN: 1559-0755
DOI: https://doi.org/10.1007/s12026-007-0022-2

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