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Comment
. 2015 Aug 24;212(9):1342-3.
doi: 10.1084/jem.2129insight4.

Inborn errors underlying herpes simplex encephalitis: From TLR3 to IRF3

Affiliations
Comment

Inborn errors underlying herpes simplex encephalitis: From TLR3 to IRF3

Shen-Ying Zhang et al. J Exp Med. .
No abstract available

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Figures

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Insight from Shen-Ying Zhang (left) and Jean-Laurent Casanova (right)
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Mutations in six genes (TLR3, UNC93B1, TRIF, TRAF3, TBK1, and IRF3), all involved in the TLR3 signaling pathway, have been found in HSE patients. TLR3, UNC93B1, and TRIF are specifically involved in TLR3 signaling. TRAF3, TBK1, and IRF3 are also involved in signaling of other RNA and DNA sensors (RIG-I, MDA5, and cGAS). TLR3 signaling is initiated by the recognition of dsRNA, inducing activation of the IRF3 and NF-κB pathways via TRIF, leading to the production of IFN-α/β and/or IFN-λ. TLR3, UNC-93B, TRIF, TRAF3, TBK1, and IRF3 deficiencies are associated with impaired IFN-α/β and/or IFN-λ production upon stimulation of TLR3 or infection with HSV-1. A mutation in the gene encoding IRF3 is described in the paper by Andersen et al.

Comment on

  • Functional IRF3 deficiency in a patient with herpes simplex encephalitis.
    Andersen LL, Mørk N, Reinert LS, Kofod-Olsen E, Narita R, Jørgensen SE, Skipper KA, Höning K, Gad HH, Østergaard L, Ørntoft TF, Hornung V, Paludan SR, Mikkelsen JG, Fujita T, Christiansen M, Hartmann R, Mogensen TH. Andersen LL, et al. J Exp Med. 2015 Aug 24;212(9):1371-9. doi: 10.1084/jem.20142274. Epub 2015 Jul 27. J Exp Med. 2015. PMID: 26216125 Free PMC article.

References

    1. Andersen, L.L., et al. ., 2015. J. Exp. Med. 10.1084/jem.20142274 - DOI - PubMed

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