Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency

Casrouge, Armanda; Zhang, Shen-Ying; Eidenschenk, Céline; Jouanguy, Emmanuelle; Puel, Anne; Yang, Kun; Alcaïs, Alexandre; Pïcard, Capucine; Mahfoufi, Nora; Nicolas, Nathalie; Lorenzo, Lazaro; Plancoulaine, Sabine; Sénéchal, Brigitte; Geissmann, Frédéric; Tabeta, Koichi; Hoebe, Kasper; Du, Xin; Miller, Richard L.; Héron, Bénédicte; Mignot, Cyril; Villemeur, Thierry Billette de; Lebon, Pierre; Dulac, Olivier; Rozenberg, Flore; Beutler, Bruce; Tardieu, Marc; Abel, Laurent; Casanova, Jean‐Laurent

doi:10.1126/science.1128346

Cited by 680 publications

(622 citation statements)

References 25 publications

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“…Indeed, there was no evidence for specific BoHV-1 DNA restriction patterns associated to these strains isolated from cattle brains whereas the DNA restriction patterns showed clear differences between BoHV-1 and the neuropathogenic strains belonging to BoHV-5 [40,132]. But several host factors may account for increased susceptibility to CNS infection by BoHV-1 as suggested by recent data obtained on sporadic cases of encephalitis caused by HSV-1 in humans [26]. For example, infants possessing homozygous deficiencies in STAT1, a key regulator protein of the antiviral pathway induced by interferons (IFN) α and β, were shown to be very susceptible to lethal CNS herpesvirus infection [28,42].…”

Section: Neuroinvasionmentioning

confidence: 99%

Bovine herpesvirus 1 infection and infectious bovine rhinotracheitis

et al. 2007

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-Bovine herpesvirus 1 (BoHV-1), classified as an alphaherpesvirus, is a major pathogen of cattle. Primary infection is accompanied by various clinical manifestations such as infectious bovine rhinotracheitis, abortion, infectious pustular vulvovaginitis, and systemic infection in neonates. When animals survive, a life-long latent infection is established in nervous sensory ganglia. Several reactivation stimuli can lead to viral re-excretion, which is responsible for the maintenance of BoHV-1 within a cattle herd. This paper focuses on an updated pathogenesis based on a molecular characterization of BoHV-1 and the description of the virus cycle. Special emphasis is accorded to the impact of the latency and reactivation cycle on the epidemiology and the control of BoHV-1. Several European countries have initiated BoHV-1 eradication schemes because of the significant losses incurred by disease and trading restrictions. The vaccines used against BoHV-1 are described in this context where the differentiation of infected from vaccinated animals is of critical importance to achieve BoHV-1 eradication.

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Section: Neuroinvasionmentioning

confidence: 99%

Bovine herpesvirus 1 infection and infectious bovine rhinotracheitis

et al. 2007

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“…It has been proposed that the weak yet detectable TRAF3 expression in this individual account for survival into adulthood [99]. Other TLRs that are implicated in conferring protective immunity against HSE in humans, are TLR 7, 8 and 9, since an autosomal recessive mutation in UNC93B, which is required for the proper signalling of these TLRs, also predisposed to HSE [100] (Table 3). …”

Section: Tlrs and Herpes Simplex Encephalitismentioning

confidence: 99%

Evaluating the role of Toll-like receptors in diseases of the central nervous system

Carty

Bowie

2011

Biochemical Pharmacology

134

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A key part of the innate immune system is a network of pattern recognition receptors (PRRs) and their associated intracellular signalling pathways. Toll-like receptors (TLRs) are one such group of PRRs that detect pathogen associated molecular patterns (PAMPs). Activation of the TLRs with their respective agonists results in the activation of intracellular signalling pathways leading to the expression of proinflammatory mediators and anti-microbial effector molecules. Activation of the innate immune system through TLRs also triggers the adaptive immune response, resulting in a comprehensive immune program to eradicate invading pathogens. It is now known that immune surveillance and inflammatory responses occur in the central nervous system (CNS). Furthermore it is becoming increasingly clear that TLRs have a role in such CNS responses andare also implicated in the pathogenesis of a number of conditions in the CNS, such as Alzheimer's, stroke and multiple sclerosis. This is likely due to the generation of endogenous TLR agonists in these conditions which amplifies a detrimental neurotoxic inflammatory response. However TLRs in some situations can be neuroprotective, if triggered in a favourable context. This review aims to examine the recent literature on TLRs in the CNS thus demonstrating their importance in a range of infectious and non-infectious diseases of the brain.

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“…This finding led us to hypothesize that HSE in otherwise healthy children might be caused by inborn errors of antiviral IFN-α/β immunity. We analyzed genome-wide linkage data and both blood and fibroblastic responses to herpes simplex virus and various viral intermediates in children with isolated HSE from this IFN-α/β-based angle, and we discovered mutations affecting five genes governing the TLR3-mediated IFN-α/β pathway (116)(117)(118)(119)(120)(121). Another group recently identified a mutation in a sixth gene (122).…”

Section: Hse: a Genetic Diseasementioning

confidence: 99%

Severe infectious diseases of childhood as monogenic inborn errors of immunity

Casanova

2015

Proc. Natl. Acad. Sci. U.S.A.

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202

163

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This paper reviews the developments that have occurred in the field of human genetics of infectious diseases from the second half of the 20th century onward. In particular, it stresses and explains the importance of the recently described monogenic inborn errors of immunity underlying resistance or susceptibility to specific infections. The monogenic component of the genetic theory provides a plausible explanation for the occurrence of severe infectious diseases during primary infection. Over the last 20 y, increasing numbers of life-threatening infectious diseases striking otherwise healthy children, adolescents, and even young adults have been attributed to single-gene inborn errors of immunity. These studies were inspired by seminal but neglected findings in plant and animal infections. Infectious diseases typically manifest as sporadic traits because human genotypes often display incomplete penetrance (most genetically predisposed individuals remain healthy) and variable expressivity (different infections can be allelic at the same locus). Infectious diseases of childhood, once thought to be archetypal environmental diseases, actually may be among the most genetically determined conditions of mankind. This nascent and testable notion has interesting medical and biological implications.

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Herpes Simplex Virus Encephalitis in Human UNC-93B Deficiency

Cited by 680 publications

References 25 publications

Bovine herpesvirus 1 infection and infectious bovine rhinotracheitis

Bovine herpesvirus 1 infection and infectious bovine rhinotracheitis

Evaluating the role of Toll-like receptors in diseases of the central nervous system

Severe infectious diseases of childhood as monogenic inborn errors of immunity

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