Autoimmunity and Primary Immunodeficiency Disorders

被引：40

作者：

Allenspach, Eric ^{[1
,2
]}

Torgerson, Troy R. ^{[1
,2
]}

机构：

[1] Univ Washington, Sch Med, Dept Pediat, Seattle, WA 98195 USA

[2] Seattle Childrens Res Inst, 1900 9th Ave,JMB-7, Seattle, WA 98101 USA

来源：

JOURNAL OF CLINICAL IMMUNOLOGY | 2016年 / 36卷

关键词：

CTLA4; cytotoxic T lymphocyte-associated protein 4. CVID; common variable immune deficiency. IL-10; interleukin-10; IPEX; immune dysregulation; polyendocrinopathy; enteropathy; X-linked. NF-kappa B; nuclear factor-kappa binding. PIDD; primary immunodeficiency disorders. STAT; signal transducer and activator of transcription. Treg; regulatory T cell; REGULATORY T-CELLS; ANHIDROTIC ECTODERMAL DYSPLASIA; NF-KAPPA-B; IMMUNE DYSREGULATION; GERMLINE MUTATIONS; 11; CARD11; DEFICIENCY; DISEASE; LUPUS; GENE;

D O I：

10.1007/s10875-016-0294-1

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Advances in DNA sequencing technologies have led to a quickening in the pace at which new genetic immunodeficiency disorders have been identified. Among the newly identified defects are a number of disorders that present primarily with autoimmunity as opposed to recurrent infections. These Bimmune dysregulation<^> disorders have begun to cluster together to form an increased understanding of some of the basic molecular mechanisms that underlie the establishment and maintenance of immune tolerance and the development of autoimmunity. This review will present three major themes that have emerged in our understanding of the mechanisms that underlie autoimmunity and immune dysregulation in humans.

引用

页码：S57 / S67

页数：11

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