Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency
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.
- Publication:
-
Science
- Pub Date:
- October 2006
- DOI:
- 10.1126/science.1128346
- Bibcode:
- 2006Sci...314..308C
- Keywords:
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- MEDICINE