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

被引：132

作者：

Ochs, Hans D.

Gambineri, Eleonora

Torgerson, Troy R.

机构：

[1] Childrens Hosp, Div Immunol Infect Dis Rheumatol, Seattle, WA 98109 USA

[2] Univ Florence, Amma Meyer Childrens Hosp, Dept Pediat, I-50132 Florence, Italy

[3] Univ Washington, Seattle, WA 98109 USA

来源：

IMMUNOLOGIC RESEARCH | 2007年 / 38卷 / 1-3期

关键词：

forkhead/winged-helix protein (FOXP3); immune dysregulation; polyendocrinopathy; enteropathy; X-linked (IPEX); stem cell transplantation; immunosuppressive therapy; scurfy mouse regulatory T cells;

D O I：

10.1007/s12026-007-0022-2

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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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收藏

页码：112 / 121

页数：10

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