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Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is the mostcommon form of sporadic viral encephalitis in western countries.Its pathogenesis remains unclear, as it affects otherwise healthypatients and only a small minority of HSV-1–infected individuals.Here, we elucidate a genetic etiology for HSE in two childrenwith autosomal recessive deficiency in the intracellular proteinUNC-93B, resulting in impaired cellular interferon-/ßand - antiviral responses. HSE can result from a single-geneimmunodeficiency that does not compromise immunity to most pathogens,unlike most known primary immunodeficiencies. Other severe infectiousdiseases may also reflect monogenic disorders of immunity.
1 Laboratoire de Génétique Humaine des Maladies Infectieuses, Université de Paris René Descartes, INSERM, U550, Faculté de Médecine Necker, Paris 75015, France. 2 French-Chinese Laboratory of Genomics and Life Sciences, Rui-jin Hospital, Shanghai Jiaotong University, Shanghai 200025, China. 3 Centre d'Etude des Déficits Immunitaires, Hôpital Necker, Paris 75015, France. 4 Laboratoire d'Etude des Cellules Mononucléées, Hôpital Necker, Paris 75015, France. 5 Scripps Research Institute, La Jolla, CA 92037, USA. 6 3M Center, St. Paul, MN 55144, USA. 7 Service de Neurologie Pédiatrique, Hôpital Trousseau, Paris 75012, France. 8 Service de Virologie, Hôpital Saint-Vincent de Paul, Paris 75014, France. 9 Service de Neurologie Pédiatrique, Hôpital Necker, Paris 75015, France. 10 Service de Neurologie Pédiatrique et INSERM U802, Hôpital de Bicêtre, Kremlin-Bicêtre 94270, France. 11 Unité d'Immunologie et d'Hématologie Pédiatriques, Hôpital Necker, Paris 75015, France.
* These authors contributed equally to this work.
Present address: Niigata University Graduate School of Medicaland Dental Sciences, Niigata 951-8514, Japan.
To whom correspondence should be addressed. E-mail: casanova{at}necker.fr
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/ß and -
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. 
Present address: Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8514, Japan. 
To whom correspondence should be addressed. E-mail: 
