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

Details

Serval ID
serval:BIB_568322E0BDD0
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Herpes simplex encephalitis in adult patients with MASP-2 deficiency.
Journal
PLoS pathogens
Author(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
Publication state
Published
Issued date
12/2019
Peer-reviewed
Oui
Volume
15
Number
12
Pages
e1008168
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
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
Yes
Create date
03/01/2020 14:23
Last modification date
08/02/2020 7:17
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