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Cited by 725

The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.

Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency

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 L., Héron, Bénédicte, Mignot, Cyril, de Villemeur, Thierry Billette, Lebon, Pierre, Dulac, Olivier, Rozenberg, Flore, Beutler, Bruce, Tardieu, Marc, Abel, Laurent, Casanova, Jean-Laurent·2006·Science
DOI 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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Crossref · 10.1126/science.1128346

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Research works printed but not linked: 28

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Metadata

Authors
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., Mignot, C., de Villemeur, T. B., Lebon, P., Dulac, O., Rozenberg, F., Beutler, B., Tardieu, M., Abel, L., & Casanova, J.-L.
Affiliations
Laboratoire de Génétique Humaine des Maladies Infectieuses, Université de Paris René Descartes, INSERM, U550, Faculté de Médecine Necker, Paris 75015, France.
Venue
Science
Publisher
American Association for the Advancement of Science (AAAS)
Published
2006-10-13
Volume
314
Issue
5797
Pages
308-312
ISSN
  • 0036-8075
  • 1095-9203
DOI
10.1126/science.1128346
Cited by
725
References
1
Language
en

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