Herpes simplex encephalitis in adult patients with MASP-2 deficiency.

Détails

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Etat: Public
Version: Final published version
Licence: CC BY 4.0
ID Serval
serval:BIB_568322E0BDD0
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Herpes simplex encephalitis in adult patients with MASP-2 deficiency.
Périodique
PLoS pathogens
Auteur(s)
Bibert S., Piret J., Quinodoz M., Collinet E., Zoete V., Michielin O., Menasria R., Meylan P., Bihl T., Erard V., Fellmann F., Rivolta C., Boivin G., Bochud P.Y.
ISSN
1553-7374 (Electronic)
ISSN-L
1553-7366
Statut éditorial
Publié
Date de publication
12/2019
Peer-reviewed
Oui
Volume
15
Numéro
12
Pages
e1008168
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: epublish
Résumé
We report here two cases of Herpes simplex virus encephalitis (HSE) in adult patients with very rare, previously uncharacterized, non synonymous heterozygous G634R and R203W substitution in mannan-binding lectin serine protease 2 (MASP2), a gene encoding a key protease of the lectin pathway of the complement system. None of the 2 patients had variants in genes involved in the TLR3-interferon signaling pathway. Both MASP2 variants induced functional defects in vitro, including a reduced (R203W) or abolished (G634R) protein secretion, a lost capability to cleave MASP-2 precursor into its active form (G634R) and an in vivo reduced antiviral activity (G634R). In a murine model of HSE, animals deficient in mannose binding lectins (MBL, the main pattern recognition molecule associated with MASP-2) had a decreased survival rate and an increased brain burden of HSV-1 compared to WT C57BL/6J mice. Altogether, these data suggest that MASP-2 deficiency can increase susceptibility to adult HSE.
Pubmed
Web of science
Open Access
Oui
Création de la notice
03/01/2020 13:23
Dernière modification de la notice
30/04/2021 6:10
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