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 Mignot; Thierry Billette De Villemeur; Pierre Lebon; Olivier Dulac; Flore Rozenberg; Bruce Beutler; Marc Tardieu; Laurent Abel; Jean Laurent Casanova

doi:10.1126/science.1128346

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

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645 Scopus citations

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 language	English (US)
Pages (from-to)	308-312
Number of pages	5
Journal	Science
Volume	314
Issue number	5797
DOIs	https://doi.org/10.1126/science.1128346
State	Published - Oct 13 2006

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Casrouge, A., Zhang, S. Y., 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, R. L., Héron, B., ... 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

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

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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.",

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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

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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.

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Herpes simplex virus encephalitis in human UNC-93B deficiency

Abstract

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