Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency

@article{Casrouge2006HerpesSV,
  title={Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency},
  author={Armanda Casrouge and Shen-Ying Zhang and Celine 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 Sénéchal and Fr{\'e}d{\'e}ric Geissmann and Koichi Tabeta and Kasper Hoebe and Xin Du and Richard L. Miller and Bénédicte Héron and Cyril Mignot and Thierry Billette de Villemeur and Pierre Lebon and Olivier Dulac and Flore Rozenberg and Bruce Beutler and Marc Tardieu and Laurent Abel and Jean Laurent Casanova},
  journal={Science},
  year={2006},
  volume={314},
  pages={308 - 312}
}
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… Expand
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