Herpes simplex virus encephalitis in human UNC-93B deficiency

Armanda Casrouge, Shen Ying Zhang, Céline Eidenschenk, Emmanuelle Jouanguy, Anne Puel, Kun Yang, Alexandre Alcais, Capucine Picard, Nora Mahfoufi, Nathalie Nicolas, Lazaro Lorenzo, Sabine Plancoulaine, Brigitte Sénéchal, Frédéric Geissmann, Koichi Tabeta, Kasper Hoebe, Xin Du, Richard L. Miller, Bénédicte Héron, Cyril MignotThierry Billette De Villemeur, Pierre Lebon, Olivier Dulac, Flore Rozenberg, Bruce Beutler, Marc Tardieu, Laurent Abel, Jean Laurent Casanova

Research output: Contribution to journalArticle

496 Citations (Scopus)

Abstract

Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the most common form of sporadic viral encephalitis in western countries. Its pathogenesis remains unclear, as it affects otherwise healthy patients and only a small minority of HSV-1-infected individuals. Here, we elucidate a genetic etiology for HSE in two children with autosomal recessive deficiency in the intracellular protein UNC-93B, resulting in impaired cellular interferon-α/β and -λ antiviral responses. HSE can result from a single-gene immunodeficiency that does not compromise immunity to most pathogens, unlike most known primary immunodeficiencies. Other severe infectious diseases may also reflect monogenic disorders of immunity.

Original languageEnglish (US)
Pages (from-to)308-312
Number of pages5
JournalScience
Volume314
Issue number5797
DOIs
StatePublished - Oct 13 2006

Fingerprint

Herpes Simplex Encephalitis
Human Herpesvirus 1
Simplexvirus
Encephalitis
Immunity
Viral Encephalitis
Interferons
Antiviral Agents
Communicable Diseases
Genes
Proteins

ASJC Scopus subject areas

  • General

Cite this

Casrouge, A., Zhang, S. Y., Eidenschenk, C., Jouanguy, E., Puel, A., Yang, K., ... Casanova, J. L. (2006). Herpes simplex virus encephalitis in human UNC-93B deficiency. Science, 314(5797), 308-312. https://doi.org/10.1126/science.1128346

Herpes simplex virus encephalitis in human UNC-93B deficiency. / Casrouge, Armanda; Zhang, Shen Ying; Eidenschenk, Céline; Jouanguy, Emmanuelle; Puel, Anne; Yang, Kun; Alcais, Alexandre; Picard, Capucine; Mahfoufi, Nora; Nicolas, Nathalie; Lorenzo, Lazaro; Plancoulaine, Sabine; Sénéchal, Brigitte; Geissmann, Frédéric; Tabeta, Koichi; Hoebe, Kasper; Du, Xin; Miller, Richard L.; Héron, Bénédicte; Mignot, Cyril; Billette De Villemeur, Thierry; Lebon, Pierre; Dulac, Olivier; Rozenberg, Flore; Beutler, Bruce; Tardieu, Marc; Abel, Laurent; Casanova, Jean Laurent.

In: Science, Vol. 314, No. 5797, 13.10.2006, p. 308-312.

Research output: Contribution to journalArticle

Casrouge, A, Zhang, SY, Eidenschenk, C, Jouanguy, E, Puel, A, Yang, K, Alcais, A, Picard, C, Mahfoufi, N, Nicolas, N, Lorenzo, L, Plancoulaine, S, Sénéchal, B, Geissmann, F, Tabeta, K, Hoebe, K, Du, X, Miller, RL, Héron, B, Mignot, C, Billette De Villemeur, T, Lebon, P, Dulac, O, Rozenberg, F, Beutler, B, Tardieu, M, Abel, L & Casanova, JL 2006, 'Herpes simplex virus encephalitis in human UNC-93B deficiency', Science, vol. 314, no. 5797, pp. 308-312. https://doi.org/10.1126/science.1128346
Casrouge A, Zhang SY, Eidenschenk C, Jouanguy E, Puel A, Yang K et al. Herpes simplex virus encephalitis in human UNC-93B deficiency. Science. 2006 Oct 13;314(5797):308-312. https://doi.org/10.1126/science.1128346
Casrouge, Armanda ; Zhang, Shen Ying ; Eidenschenk, Céline ; Jouanguy, Emmanuelle ; Puel, Anne ; Yang, Kun ; Alcais, Alexandre ; Picard, Capucine ; Mahfoufi, Nora ; Nicolas, Nathalie ; Lorenzo, Lazaro ; Plancoulaine, Sabine ; Sénéchal, Brigitte ; Geissmann, Frédéric ; Tabeta, Koichi ; Hoebe, Kasper ; Du, Xin ; Miller, Richard L. ; Héron, Bénédicte ; Mignot, Cyril ; Billette De Villemeur, Thierry ; Lebon, Pierre ; Dulac, Olivier ; Rozenberg, Flore ; Beutler, Bruce ; Tardieu, Marc ; Abel, Laurent ; Casanova, Jean Laurent. / Herpes simplex virus encephalitis in human UNC-93B deficiency. In: Science. 2006 ; Vol. 314, No. 5797. pp. 308-312.
@article{6cec2254c65f40b985e991cd4efe2c4c,
title = "Herpes simplex virus encephalitis in human UNC-93B deficiency",
abstract = "Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the most common form of sporadic viral encephalitis in western countries. Its pathogenesis remains unclear, as it affects otherwise healthy patients and only a small minority of HSV-1-infected individuals. Here, we elucidate a genetic etiology for HSE in two children with autosomal recessive deficiency in the intracellular protein UNC-93B, resulting in impaired cellular interferon-α/β and -λ antiviral responses. HSE can result from a single-gene immunodeficiency that does not compromise immunity to most pathogens, unlike most known primary immunodeficiencies. Other severe infectious diseases may also reflect monogenic disorders of immunity.",
author = "Armanda Casrouge and Zhang, {Shen Ying} and C{\'e}line Eidenschenk and Emmanuelle Jouanguy and Anne Puel and Kun Yang and Alexandre Alcais and Capucine Picard and Nora Mahfoufi and Nathalie Nicolas and Lazaro Lorenzo and Sabine Plancoulaine and Brigitte S{\'e}n{\'e}chal and Fr{\'e}d{\'e}ric Geissmann and Koichi Tabeta and Kasper Hoebe and Xin Du and Miller, {Richard L.} and B{\'e}n{\'e}dicte H{\'e}ron and Cyril Mignot and {Billette De Villemeur}, Thierry and Pierre Lebon and Olivier Dulac and Flore Rozenberg and Bruce Beutler and Marc Tardieu and Laurent Abel and Casanova, {Jean Laurent}",
year = "2006",
month = "10",
day = "13",
doi = "10.1126/science.1128346",
language = "English (US)",
volume = "314",
pages = "308--312",
journal = "Science",
issn = "0036-8075",
publisher = "American Association for the Advancement of Science",
number = "5797",

}

TY - JOUR

T1 - Herpes simplex virus encephalitis in human UNC-93B deficiency

AU - Casrouge, Armanda

AU - Zhang, Shen Ying

AU - Eidenschenk, Céline

AU - Jouanguy, Emmanuelle

AU - Puel, Anne

AU - Yang, Kun

AU - Alcais, Alexandre

AU - Picard, Capucine

AU - Mahfoufi, Nora

AU - Nicolas, Nathalie

AU - Lorenzo, Lazaro

AU - Plancoulaine, Sabine

AU - Sénéchal, Brigitte

AU - Geissmann, Frédéric

AU - Tabeta, Koichi

AU - Hoebe, Kasper

AU - Du, Xin

AU - Miller, Richard L.

AU - Héron, Bénédicte

AU - Mignot, Cyril

AU - Billette De Villemeur, Thierry

AU - Lebon, Pierre

AU - Dulac, Olivier

AU - Rozenberg, Flore

AU - Beutler, Bruce

AU - Tardieu, Marc

AU - Abel, Laurent

AU - Casanova, Jean Laurent

PY - 2006/10/13

Y1 - 2006/10/13

N2 - Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the most common form of sporadic viral encephalitis in western countries. Its pathogenesis remains unclear, as it affects otherwise healthy patients and only a small minority of HSV-1-infected individuals. Here, we elucidate a genetic etiology for HSE in two children with autosomal recessive deficiency in the intracellular protein UNC-93B, resulting in impaired cellular interferon-α/β and -λ antiviral responses. HSE can result from a single-gene immunodeficiency that does not compromise immunity to most pathogens, unlike most known primary immunodeficiencies. Other severe infectious diseases may also reflect monogenic disorders of immunity.

AB - Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the most common form of sporadic viral encephalitis in western countries. Its pathogenesis remains unclear, as it affects otherwise healthy patients and only a small minority of HSV-1-infected individuals. Here, we elucidate a genetic etiology for HSE in two children with autosomal recessive deficiency in the intracellular protein UNC-93B, resulting in impaired cellular interferon-α/β and -λ antiviral responses. HSE can result from a single-gene immunodeficiency that does not compromise immunity to most pathogens, unlike most known primary immunodeficiencies. Other severe infectious diseases may also reflect monogenic disorders of immunity.

UR - http://www.scopus.com/inward/record.url?scp=33750016788&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33750016788&partnerID=8YFLogxK

U2 - 10.1126/science.1128346

DO - 10.1126/science.1128346

M3 - Article

C2 - 16973841

AN - SCOPUS:33750016788

VL - 314

SP - 308

EP - 312

JO - Science

JF - Science

SN - 0036-8075

IS - 5797

ER -