Herpes simplex virus encephalitis in human UNC-93B deficiency
Science, 2006•science.org
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 …
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 …
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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