TLR3 deficiency in patients with herpes simplex encephalitis

Shen Ying Zhang; Emmanuelle Jouanguy; Sophie Ugolini; Asma Smahi; Gaëlle Elain; Pedro Romero; David Segal; Vanessa Sancho-Shimizu; 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 Casanova

doi:10.1126/science.1139522

TLR3 deficiency in patients with herpes simplex encephalitis

Shen Ying Zhang, Emmanuelle Jouanguy, Sophie Ugolini, Asma Smahi, Gaëlle Elain, Pedro Romero, David Segal, Vanessa Sancho-Shimizu, 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 BarreiroJoshua 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 Casanova^*

^*Corresponding author for this work

Research output: Contribution to journal › Journal Article › peer-review

935 Scopus citations

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.

Original language	English
Pages (from-to)	1522-1527
Number of pages	6
Journal	Science
Volume	317
Issue number	5844
DOIs	https://doi.org/10.1126/science.1139522
State	Published - 14 09 2007
Externally published	Yes

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Zhang, S. Y., Jouanguy, E., Ugolini, S., Smahi, A., Elain, G., Romero, P., Segal, D., Sancho-Shimizu, V., Lorenzo, L., Puel, A., Picard, C., Chapgier, A., Plancoulaine, S., Titeux, M., Cognet, C., Von Bernuth, H., Ku, C. L., Casrouge, A., Zhang, X. X., ... Casanova, J. L. (2007). TLR3 deficiency in patients with herpes simplex encephalitis. Science, 317(5844), 1522-1527. https://doi.org/10.1126/science.1139522

@article{a2d4645a562b4afb9af0bbec83b7de98,

title = "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.",

author = "Zhang, {Shen Ying} and Emmanuelle Jouanguy and Sophie Ugolini and Asma Smahi and Ga{\"e}lle Elain and Pedro Romero and David Segal and Vanessa Sancho-Shimizu and Lazaro Lorenzo and Anne Puel and Capucine Picard and Ariane Chapgier and Sabine Plancoulaine and Matthias Titeux and C{\'e}line Cognet and {Von Bernuth}, Horst and Ku, {Cheng Lung} and Armanda Casrouge and Zhang, {Xin Xin} and Luis Barreiro and Joshua Leonard and Claire Hamilton and Pierre Lebon and B{\'e}n{\'e}dicte H{\'e}ron and Louis Vall{\'e}e and Lluis Quintana-Murci and Alain Hovnanian and Flore Rozenberg and Eric Vivier and Fr{\'e}d{\'e}ric Geissmann and Marc Tardieu and Laurent Abel and Casanova, {Jean Laurent}",

year = "2007",

month = sep,

day = "14",

doi = "10.1126/science.1139522",

language = "英语",

volume = "317",

pages = "1522--1527",

journal = "Science",

issn = "0036-8075",

publisher = "American Association for the Advancement of Science",

number = "5844",

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Zhang, SY, Jouanguy, E, Ugolini, S, Smahi, A, Elain, G, Romero, P, Segal, D, Sancho-Shimizu, V, Lorenzo, L, Puel, A, Picard, C, Chapgier, A, Plancoulaine, S, Titeux, M, Cognet, C, Von Bernuth, H, Ku, CL, Casrouge, A, Zhang, XX, Barreiro, L, Leonard, J, Hamilton, C, Lebon, P, Héron, B, Vallée, L, Quintana-Murci, L, Hovnanian, A, Rozenberg, F, Vivier, E, Geissmann, F, Tardieu, M, Abel, L & Casanova, JL 2007, 'TLR3 deficiency in patients with herpes simplex encephalitis', Science, vol. 317, no. 5844, pp. 1522-1527. https://doi.org/10.1126/science.1139522

TY - JOUR

T1 - TLR3 deficiency in patients with herpes simplex encephalitis

AU - Zhang, Shen Ying

AU - Jouanguy, Emmanuelle

AU - Ugolini, Sophie

AU - Smahi, Asma

AU - Elain, Gaëlle

AU - Romero, Pedro

AU - Segal, David

AU - Sancho-Shimizu, Vanessa

AU - Lorenzo, Lazaro

AU - Puel, Anne

AU - Picard, Capucine

AU - Chapgier, Ariane

AU - Plancoulaine, Sabine

AU - Titeux, Matthias

AU - Cognet, Céline

AU - Von Bernuth, Horst

AU - Ku, Cheng Lung

AU - Casrouge, Armanda

AU - Zhang, Xin Xin

AU - Barreiro, Luis

AU - Leonard, Joshua

AU - Hamilton, Claire

AU - Lebon, Pierre

AU - Héron, Bénédicte

AU - Vallée, Louis

AU - Quintana-Murci, Lluis

AU - Hovnanian, Alain

AU - Rozenberg, Flore

AU - Vivier, Eric

AU - Geissmann, Frédéric

AU - Tardieu, Marc

AU - Abel, Laurent

AU - Casanova, Jean Laurent

PY - 2007/9/14

Y1 - 2007/9/14

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=34548699323&partnerID=8YFLogxK

U2 - 10.1126/science.1139522

DO - 10.1126/science.1139522

M3 - 文章

C2 - 17872438

AN - SCOPUS:34548699323

SN - 0036-8075

VL - 317

SP - 1522

EP - 1527

JO - Science

JF - Science

IS - 5844

ER -

TLR3 deficiency in patients with herpes simplex encephalitis

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