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

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

ASJC Scopus subject areas

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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