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Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity
by
Shen-Ying, Zhang
in
Antiviral drugs
/ Brain stem
/ Central nervous system
/ Childhood
/ Children
/ Encephalitis
/ Forebrain
/ Herpes simplex
/ Herpes viruses
/ Immunity
/ Immunology
/ Nervous system
/ TLR3 protein
/ Toll-like receptors
/ α-Interferon
2020
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Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity
by
Shen-Ying, Zhang
in
Antiviral drugs
/ Brain stem
/ Central nervous system
/ Childhood
/ Children
/ Encephalitis
/ Forebrain
/ Herpes simplex
/ Herpes viruses
/ Immunity
/ Immunology
/ Nervous system
/ TLR3 protein
/ Toll-like receptors
/ α-Interferon
2020
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Do you wish to request the book?
Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity
by
Shen-Ying, Zhang
in
Antiviral drugs
/ Brain stem
/ Central nervous system
/ Childhood
/ Children
/ Encephalitis
/ Forebrain
/ Herpes simplex
/ Herpes viruses
/ Immunity
/ Immunology
/ Nervous system
/ TLR3 protein
/ Toll-like receptors
/ α-Interferon
2020
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Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity
Journal Article
Herpes simplex virus encephalitis of childhood: inborn errors of central nervous system cell-intrinsic immunity
2020
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Overview
Herpes simplex virus 1 (HSV-1) encephalitis (HSE) is the most common sporadic viral encephalitis in Western countries. Over the last 15 years, human genetic and immunological studies have provided proof-of-principle that childhood HSE can result from inborn errors of central nervous system (CNS)-specific, cell-intrinsic immunity to HSV-1. HSE-causing mutations of eight genes disrupt known (TLR3-dependent IFN-α/β immunity) and novel (dependent on DBR1 or snoRNA31) antiviral mechanisms. Monogenic inborn errors confer susceptibility to forebrain (TLR3-IFN or snoRNA31) or brainstem (DBR1) HSE. Most of these disorders display incomplete clinical penetrance, with the possible exception of DBR1 deficiency. They account for a small, but non-negligible proportion of cases (about 7%). These findings pave the way for the gradual definition of the genetic and immunological architecture of childhood HSE, with both biological and clinical implications.
Publisher
Springer Nature B.V
Subject
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