TLR3 deficiency in patients with herpes simplex encephalitis.

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Zhang, Shen-Ying | Jouanguy, Emmanuelle | Ugolini, Sophie | Smahi, Asma | Elain, Gaëlle | Romero, Pedro | Segal, David | Sancho-Shimizu, Vanessa | Lorenzo, Lazaro | Puel, Anne | Picard, Capucine | Chapgier, Ariane | Plancoulaine, Sabine | Titeux, Matthias | Cognet, Céline | von Bernuth, Horst | Ku, Cheng-Lung | Casrouge, Armanda | Zhang, Xin-Xin | Barreiro, Luis | Leonard, Joshua | Hamilton, Claire | Lebon, Pierre | Héron, Bénédicte | Vallée, Louis | Quintana-Murci, Lluis | Hovnanian, Alain | Rozenberg, Flore | Vivier, Eric | Geissmann, Frédéric | Tardieu, Marc | Abel, Laurent | Casanova, Jean-Laurent

Edité par CCSD ; American Association for the Advancement of Science (AAAS) -

International audience. 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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