Lighting the fires within: the cell biology of autoinflammatory diseases

被引:132
作者
Park, Heiyoung [1 ]
Bourla, Ariel Bulua [1 ]
Kastner, Daniel L. [2 ]
Colbert, Robert A. [3 ]
Siegel, Richard M. [1 ]
机构
[1] Natl Inst Arthrit & Musculoskeletal & Skin Dis NI, Immunoregulat Sect, Autoimmun Branch, NIH, Bethesda, MD USA
[2] NHGRI, Inflammatory Dis Sect, Med Genet Branch, NIH, Bethesda, MD 20892 USA
[3] NIAMS, Pediat Translat Res Branch, NIH, Bethesda, MD USA
基金
美国国家卫生研究院;
关键词
ENDOPLASMIC-RETICULUM STRESS; FAMILIAL MEDITERRANEAN FEVER; UNFOLDED PROTEIN RESPONSE; JUVENILE IDIOPATHIC ARTHRITIS; NALP3; INFLAMMASOME; NLRP3; TNF RECEPTOR; TRANSCRIPTION FACTOR; PERIODIC SYNDROME; ER STRESS;
D O I
10.1038/nri3261
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Autoinflammatory diseases are characterized by seemingly unprovoked pathological activation of the innate immune system in the absence of autoantibodies or autoreactive T cells. Discovery of the causative mutations underlying several monogenic autoinflammatory diseases has identified key regulators of innate immune responses. Recent studies have highlighted the role of misfolding, oligomerization and abnormal trafficking of pathogenic mutant proteins in triggering autoinflammation, and suggest that more common rheumatic diseases may have an autoinflammatory component. This coincides with recent discoveries of new links between endoplasmic reticulum stress and inflammatory signalling pathways, which support the emerging view that autoinflammatory diseases may be due to pathological dysregulation of stress-sensing pathways that normally function in host defence.
引用
收藏
页码:570 / 580
页数:11
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