| First Author | Bibert S | Year | 2019 |
| Journal | PLoS Pathog | Volume | 15 |
| Issue | 12 | Pages | e1008168 |
| PubMed ID | 31869396 | Mgi Jnum | J:292499 |
| Mgi Id | MGI:6448690 | Doi | 10.1371/journal.ppat.1008168 |
| Citation | Bibert S, et al. (2019) Herpes simplex encephalitis in adult patients with MASP-2 deficiency. PLoS Pathog 15(12):e1008168 |
| abstractText | 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. |
