Editors' ChoiceGENETICS

Rethinking Viral Resistance

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Science's STKE  17 Oct 2006:
Vol. 2006, Issue 357, pp. tw361
DOI: 10.1126/stke.3572006tw361

In their investigations of herpes simplex encephalitis, Casrouge et al. found that a single gene defect can impair resistance to the causative herpes simplex virus but not others. The rare autosomal recessive mutation was present in a conserved gene encoding the endoplasmic reticulum membrane protein UNC-93B, which is thought to be involved in the cellular antiviral immune response. Cells from these subjects displayed defective response of some interferon genes, which would explain the children’s severe defect in controlling viral replication. In contrast, the seemingly robust immunity of the subjects to other pathogens suggests, rather unexpectedly, that a single gene defect can lead to a pathogen-specific form of immunodeficiency.

A. Casrouge, S.-Y. Zhang, C. Eidenschenk, E. Jouanguy, A. Puel, K. Yang, A. Alcais, C. Picard, N. Mahfoufi, N. Nicolas, L. Lorenzo, S. Plancoulaine, B. Sénéchal, F. Geissmann, K. Tabeta, K. Hoebe, X. Du, R. L. Miller, B. Héron, C. Mignot, T. B. de Villemeur, P. Lebon, O. Dulac, F. Rozenberg, B. Beutler, M. Tardieu, L. Abel, J.-L. Casanova, Herpes simplex virus encephalitis in human UNC-93B deficiency. Science 314, 308-312 (2006). [Abstract] [Full Text]

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