論文

査読有り 国際誌
2006年10月13日

Herpes simplex virus encephalitis in human UNC-93B deficiency.

Science (New York, N.Y.)
  • 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 Mignot
  • Thierry Billette de Villemeur
  • Pierre Lebon
  • Olivier Dulac
  • Flore Rozenberg
  • Bruce Beutler
  • Marc Tardieu
  • Laurent Abel
  • Jean-Laurent Casanova
  • 全て表示

314
5797
開始ページ
308
終了ページ
12
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1126/science.1128346
出版者・発行元
AMER ASSOC ADVANCEMENT SCIENCE

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-alpha/beta and -lambda 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.

リンク情報
DOI
https://doi.org/10.1126/science.1128346
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/16973841
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000241194100044&DestApp=WOS_CPL
Scopus
https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=33750016788&origin=inward
Scopus Citedby
https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=33750016788&origin=inward
URL
http://europepmc.org/abstract/med/16973841
URL
http://orcid.org/0000-0002-7019-1872
ID情報
  • DOI : 10.1126/science.1128346
  • ISSN : 0036-8075
  • eISSN : 1095-9203
  • ORCIDのPut Code : 41626672
  • PubMed ID : 16973841
  • SCOPUS ID : 33750016788
  • Web of Science ID : WOS:000241194100044

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