RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.

RNF213 is the first identified susceptibility gene for moyamoya disease, and the encoded protein was recently recognized as a key antimicrobial protein. However, the function of RNF213 in host defense against brain infection remains unclear. Here, we show that increased expression of Rnf213 is signi...

Full description

Saved in:
Bibliographic Details
Main Authors: Yanfeng Li, Qingqing Xie, Luyu Yang, Qian Jiang, Zhiping Liu, Chengjiang Gao, Xiaopeng Qi, Tao Xu
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2025-07-01
Series:PLoS Pathogens
Online Access:https://doi.org/10.1371/journal.ppat.1013333
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850099386002964480
author Yanfeng Li
Qingqing Xie
Luyu Yang
Qian Jiang
Zhiping Liu
Chengjiang Gao
Xiaopeng Qi
Tao Xu
author_facet Yanfeng Li
Qingqing Xie
Luyu Yang
Qian Jiang
Zhiping Liu
Chengjiang Gao
Xiaopeng Qi
Tao Xu
author_sort Yanfeng Li
collection DOAJ
description RNF213 is the first identified susceptibility gene for moyamoya disease, and the encoded protein was recently recognized as a key antimicrobial protein. However, the function of RNF213 in host defense against brain infection remains unclear. Here, we show that increased expression of Rnf213 is significantly regulated by interferon alpha/beta receptor (IFNAR) signaling during bacterial infection and ligand stimulation. RNF213 deficiency impairs type I interferon (IFN-I) production and decreases the expression of interferon-stimulated genes (ISGs) in response to IFN-β stimulation and Acinetobacter baumannii infection. Mechanistically, RNF213 interacts with TRAF3 and mediates the K27-linked polyubiquitination of TRAF3 at K160. RNF213 regulates the expression of the endothelial tight junction-related genes Claudin-5, Occludin, and Pecam1 via IFN-I signaling. Furthermore, RNF213 deficiency in nonimmune cells increases blood‒brain barrier (BBB) disruption and the bacterial load in the brain parenchyma in response to A. baumannii infection due to impaired IFN-I signaling. Thus, RNF213 mediates BBB integrity by targeting TRAF3 for the regulation of IFN-I signaling against bacterial brain infection. Our study principally provides a deeper understanding of the function of RNF213 and reveals potential therapeutic targets against bacterial brain infection and moyamoya disease.
format Article
id doaj-art-5c77fda84b5f428e8c7d04cb2709c77a
institution DOAJ
issn 1553-7366
1553-7374
language English
publishDate 2025-07-01
publisher Public Library of Science (PLoS)
record_format Article
series PLoS Pathogens
spelling doaj-art-5c77fda84b5f428e8c7d04cb2709c77a2025-08-20T02:40:30ZengPublic Library of Science (PLoS)PLoS Pathogens1553-73661553-73742025-07-01217e101333310.1371/journal.ppat.1013333RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.Yanfeng LiQingqing XieLuyu YangQian JiangZhiping LiuChengjiang GaoXiaopeng QiTao XuRNF213 is the first identified susceptibility gene for moyamoya disease, and the encoded protein was recently recognized as a key antimicrobial protein. However, the function of RNF213 in host defense against brain infection remains unclear. Here, we show that increased expression of Rnf213 is significantly regulated by interferon alpha/beta receptor (IFNAR) signaling during bacterial infection and ligand stimulation. RNF213 deficiency impairs type I interferon (IFN-I) production and decreases the expression of interferon-stimulated genes (ISGs) in response to IFN-β stimulation and Acinetobacter baumannii infection. Mechanistically, RNF213 interacts with TRAF3 and mediates the K27-linked polyubiquitination of TRAF3 at K160. RNF213 regulates the expression of the endothelial tight junction-related genes Claudin-5, Occludin, and Pecam1 via IFN-I signaling. Furthermore, RNF213 deficiency in nonimmune cells increases blood‒brain barrier (BBB) disruption and the bacterial load in the brain parenchyma in response to A. baumannii infection due to impaired IFN-I signaling. Thus, RNF213 mediates BBB integrity by targeting TRAF3 for the regulation of IFN-I signaling against bacterial brain infection. Our study principally provides a deeper understanding of the function of RNF213 and reveals potential therapeutic targets against bacterial brain infection and moyamoya disease.https://doi.org/10.1371/journal.ppat.1013333
spellingShingle Yanfeng Li
Qingqing Xie
Luyu Yang
Qian Jiang
Zhiping Liu
Chengjiang Gao
Xiaopeng Qi
Tao Xu
RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
PLoS Pathogens
title RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
title_full RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
title_fullStr RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
title_full_unstemmed RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
title_short RNF213 regulates blood‒brain barrier integrity by targeting TRAF3 for type I interferon activation during A. baumannii infection.
title_sort rnf213 regulates blood brain barrier integrity by targeting traf3 for type i interferon activation during a baumannii infection
url https://doi.org/10.1371/journal.ppat.1013333
work_keys_str_mv AT yanfengli rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT qingqingxie rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT luyuyang rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT qianjiang rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT zhipingliu rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT chengjianggao rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT xiaopengqi rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection
AT taoxu rnf213regulatesbloodbrainbarrierintegritybytargetingtraf3fortypeiinterferonactivationduringabaumanniiinfection