期刊
IMMUNITY
卷 33, 期 3, 页码 400-411出版社
CELL PRESS
DOI: 10.1016/j.immuni.2010.08.014
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资金
- AXA
- Schlumberger Foundation
- BNP-Paribas Foundation
- Groupement d'Interet Scientifique Maladies Rares
- Action Concertee Incitative de Microbiologie
- March of Dimes
- Agence Nationale pour la Recherche
- Eppley Foundation
- National Institute of Allergy and Infectious Diseases [1R01AI088364]
- Thrasher Research Fund
- Jeffrey Modell Foundation
- Talecris Biotherapeutics
- St. Giles Foundation
- Rockefeller University Center for Clinical and Translational Science [5UL1RR024143]
- Rockefeller University
- European Union
Tumor necrosis factor (TNF) receptor-associated factor 3 (TRAF3) functions downstream of multiple TNF receptors and receptors that induce interferon-alpha (IFN-alpha), IFN-beta, and IFN-lambda production, including Toll-like receptor 3 (TLR3), which is deficient in some patients with herpes simplex virus-1 encephalitis (HSE). Mice lacking TRAF3 die in the neonatal period, preventing direct investigation of the role of TRAF3 in immune responses and host defenses in vivo. Here, we report autosomal dominant, human TRAF3 deficiency in a young adult with a history of HSE in childhood. The TRAF3 mutant allele is loss-of-expression, loss-of-function, dominant-negative and associated with impaired, but not abolished, TRAF3-dependent responses upon stimulation of both TNF receptors and receptors that induce IFN production. TRAF3 deficiency is associated with a clinical phenotype limited to HSE resulting from the impairment of TLR3-dependent induction of IFN. Thus, TLR3-mediated immunity against primary infection by HSV-1 in the central nervous system is critically dependent on TRAF3.
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