PMID- 37586842
OWN - NLM
STAT- MEDLINE
DCOM- 20240301
LR  - 20240308
IS  - 1750-3639 (Electronic)
IS  - 1015-6305 (Print)
IS  - 1015-6305 (Linking)
VI  - 34
IP  - 2
DP  - 2024 Mar
TI  - Increased expression of human antiviral protein MxA in FUS proteinopathy in 
      amyotrophic lateral sclerosis.
PG  - e13191
LID - 10.1111/bpa.13191 [doi]
LID - e13191
AB  - FUS mutations are one of the major mutations in familial amyotrophic lateral 
      sclerosis (ALS). The pathological hallmark is FUS-positive neuronal cytoplasmic 
      inclusions (FUS-NCI), known as FUS proteinopathy. Human myxovirus resistance 
      protein 1 (MxA) is an IFN-induced dynamin-like GTPase that acts as antiviral 
      factor. In this study, we examined the expression of MxA in neurons bearing 
      FUS-NCI. We performed immunohistochemistry for FUS and MxA to examine the 
      expression of MxA in two autopsy cases with different FUS gene mutations 
      localized at the nuclear localization signal site (Case 1, H517P; Case 2, R521C). 
      MxA. Most neurons bearing FUS-NCI have increased cytoplasmic MxA expression. 
      Increased cytoplasmic MxA showed several distribution patterns in relation to 
      FUS-NCIs such as the following: colocalization with NCI, distribution more widely 
      than NCI, and different distribution peaks from NCI. Our results suggested that 
      antiviral signaling IFNs are involved upstream in the formation of FUS-NCI in 
      ALS-FUS patients.
CI  - (c) 2023 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf 
      of International Society of Neuropathology.
FAU - Honda, Hiroyuki
AU  - Honda H
AUID- ORCID: 0000-0002-4225-7248
AD  - Department of Neuropathology, Graduate School of Medical Sciences, Kyushu 
      University, Fukuoka, Japan.
AD  - Division of Neurology, Department of Neurology, Neuro Muscular Center, National 
      Hospital Organization, Omuta National Hospital, Fukuoka, Japan.
AD  - Neuropathology Center, National Hospital Organization, Omuta National Hospital, 
      Fukuoka, Japan.
FAU - Yagita, Kaoru
AU  - Yagita K
AD  - Department of Neuropathology, Graduate School of Medical Sciences, Kyushu 
      University, Fukuoka, Japan.
FAU - Arahata, Hajime
AU  - Arahata H
AD  - Division of Neurology, Department of Neurology, Neuro Muscular Center, National 
      Hospital Organization, Omuta National Hospital, Fukuoka, Japan.
FAU - Hamasaki, Hideomi
AU  - Hamasaki H
AD  - Department of Neuropathology, Graduate School of Medical Sciences, Kyushu 
      University, Fukuoka, Japan.
FAU - Noguchi, Hideko
AU  - Noguchi H
AD  - Department of Neuropathology, Graduate School of Medical Sciences, Kyushu 
      University, Fukuoka, Japan.
FAU - Koyama, Sachiko
AU  - Koyama S
AD  - Department of Neuropathology, Graduate School of Medical Sciences, Kyushu 
      University, Fukuoka, Japan.
FAU - Sasagasako, Naokazu
AU  - Sasagasako N
AD  - Division of Neurology, Department of Neurology, Neuro Muscular Center, National 
      Hospital Organization, Omuta National Hospital, Fukuoka, Japan.
LA  - eng
PT  - Journal Article
DEP - 20230816
PL  - Switzerland
TA  - Brain Pathol
JT  - Brain pathology (Zurich, Switzerland)
JID - 9216781
RN  - 0 (Antiviral Agents)
RN  - 0 (FUS protein, human)
RN  - 0 (RNA-Binding Protein FUS)
RN  - 0 (MX1 protein, human)
SB  - IM
MH  - Humans
MH  - *Amyotrophic Lateral Sclerosis/pathology
MH  - Antiviral Agents/metabolism
MH  - Mutation
MH  - Neurons/pathology
MH  - RNA-Binding Protein FUS/genetics
PMC - PMC10901610
OTO - NOTNLM
OT  - ALS
OT  - FUS
OT  - MxA
OT  - interferon
OT  - myxovirus resistance protein
COIS- The authors have no conflicts of interests to declare.
EDAT- 2023/08/17 00:42
MHDA- 2024/03/01 06:43
PMCR- 2023/08/16
CRDT- 2023/08/16 21:38
PHST- 2022/11/18 00:00 [received]
PHST- 2023/06/06 00:00 [accepted]
PHST- 2024/03/01 06:43 [medline]
PHST- 2023/08/17 00:42 [pubmed]
PHST- 2023/08/16 21:38 [entrez]
PHST- 2023/08/16 00:00 [pmc-release]
AID - BPA13191 [pii]
AID - 10.1111/bpa.13191 [doi]
PST - ppublish
SO  - Brain Pathol. 2024 Mar;34(2):e13191. doi: 10.1111/bpa.13191. Epub 2023 Aug 16.