IPEX, FOXP3 and regulatory T-cells:: a model for autoimmunity
IPEX, FOXP3 and regulatory T-cells:: a model for autoimmunity
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DOI：
10.1007/s12026-007-0022-2
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发表时间：
2007-01-01
影响因子：
4.4
通讯作者：
Torgerson, Troy R.
中科院分区：
文献类型：
--
作者：
Ochs, Hans D.;Gambineri, Eleonora;Torgerson, Troy R.
FOXP3是胸腺中调节性T细胞发育的关键介质。自然发生的FOXP3突变会干扰这一过程，导致产生自身攻击性淋巴细胞克隆，这些克隆直接导致人类免疫失调、多内分泌病、肠病、X连锁综合征（IPEX）和小鼠皮屑病。干细胞移植是治疗IPEX患者的唯一方法。对这种罕见疾病的研究为免疫抑制，自身免疫和耐受机制提供了重要的见解，未来的研究可能会导致新的策略，不仅治疗IPEX患者，还治疗那些患有自身免疫，移植物抗宿主病或癌症的患者。
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.