TLR3 Deficiency in Patients with Herpes Simplex Encephalitis

• DOI: 10.1126/science.1139522
• Publication Date: 2007-09-13T21:09:55Z
• Authors: Shen-Ying Zhang; Emmanuelle Jouanguy; Sophie Ugolini; Asma Smahi; Gaëlle Elain; Pedro Romero; David Segal; Vanessa Sancho-Sh...; Lazaro Lorenzo; Anne Puel; Capucine Picard; Ariane Chapgier; Sabine Plancoulaine; Matthias Titeux; Céline Cognet; Horst von Bernuth; Cheng-Lung Ku; Armanda Casrouge; Xin-Xin Zhang; Luis Barreiro; Joshua Leonard; Claire Hamilton; Pierre Lebon; Bénédicte Héron; Louis Vallée; Lluis Quintana-Murci; Alain Hovnanian; Flore Rozenberg; Eric Vivier; Frédéric Geissmann; Marc Tardieu; Laurent Abel; Jean-Laurent Casa...
• Keywords: TLR3

Some Toll and Toll-like receptors (TLRs) provide immunity to experimental infections in animal models, but their contribution host defense natural ecosystems is unknown. We report a dominant-negative TLR3 allele otherwise healthy children with herpes simplex virus 1 (HSV-1) encephalitis. expressed the central nervous system (CNS), where it required control HSV-1, which spreads from epithelium CNS via cranial nerves. also epithelial dendritic cells, apparently use TLR3-independent pathways prevent further dissemination of HSV-1 resistance other pathogens TLR3-deficient patients. Human appears be redundant most microbes vital for CNS, suggests that neurotropic viruses have contributed evolutionary maintenance TLR3.