期刊
CURRENT ALLERGY AND ASTHMA REPORTS
卷 13, 期 4, 页码 361-370出版社
CURRENT MEDICINE GROUP
DOI: 10.1007/s11882-013-0350-3
关键词
Review; Autoimmunity; Primary immunodeficiency; Autoreactive; Autoantigen; Tolerance; Apoptosis; Autoimmune polyendocrinopathy candidiasis ectodermal dysplasia (APECED); Hyper IgM syndrome (HIgM); X-linked agammaglobulinemia (XLA); Common variable immunodeficiency (CVID); Immunodysregulation polyendocrinopathy enteropathy x-linked (IPEX); Omenn syndrome (OS); DiGeorge syndrome; Autoimmune lymphoproliferative syndrome (ALPS); Wiskott Aldrich syndrome (WAS); IgA deficiency (IgAD); Complement deficiency; Chronic granulomatous disease (CGD)
资金
- NIAMS NIH HHS [R03 AR059286] Funding Source: Medline
Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune phenomena in addition to having increased susceptibility to infections from their impaired immunity. Although much of our understanding comes from data gathered through experimental models, there are several well-characterized PID that have improved our knowledge of the pathways that drive autoimmunity. The goals of this review will be to discuss these immunodeficiencies and to review the literature with respect to the proposed mechanisms for autoimmunity within each put forth to date.
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