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TLR3 deficiency in patients with herpes simplex encephalitis.

Abstract : Some Toll and Toll-like receptors (TLRs) provide immunity to experimental infections in animal models, but their contribution to host defense in natural ecosystems is unknown. We report a dominant-negative TLR3 allele in otherwise healthy children with herpes simplex virus 1 (HSV-1) encephalitis. TLR3 is expressed in the central nervous system (CNS), where it is required to control HSV-1, which spreads from the epithelium to the CNS via cranial nerves. TLR3 is also expressed in epithelial and dendritic cells, which apparently use TLR3-independent pathways to prevent further dissemination of HSV-1 and to provide resistance to other pathogens in TLR3-deficient patients. Human TLR3 appears to be redundant in host defense to most microbes but is vital for natural immunity to HSV-1 in the CNS, which suggests that neurotropic viruses have contributed to the evolutionary maintenance of TLR3.
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https://hal.archives-ouvertes.fr/hal-00297308
Contributor : Marie Laure Haon <>
Submitted on : Tuesday, July 15, 2008 - 5:35:23 PM
Last modification on : Thursday, August 27, 2020 - 3:10:05 PM

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Shen-Ying Zhang, Emmanuelle Jouanguy, Sophie Ugolini, Asma Smahi, Gaëlle Elain, et al.. TLR3 deficiency in patients with herpes simplex encephalitis.. Science, American Association for the Advancement of Science, 2007, 317 (5844), pp.1522-7. ⟨10.1126/science.1139522⟩. ⟨hal-00297308⟩

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