Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency

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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 | Héron, Bénédicte | Mignot, Cyril | de Villemeur, Thierry Billette | Lebon, Pierre | Dulac, Olivier | Rozenberg, Flore | Beutler, Bruce | Tardieu, Marc | Abel, Laurent | Casanova, Jean-Laurent

Edité par CCSD ; American Association for the Advancement of Science (AAAS) -

International audience. 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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