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ID 34808
file
creator
Kawabata, Ryoko
Yoshida, Asuka
subject
Optineurin
ALS
IRF3
NDC
Medical sciences
abstract
Optineurin has been shown to be involved in primary open-angle glaucoma. We recently found that optineurin is involved in familial amyotrophic lateral sclerosis (ALS). On the other hand, optineurin has been shown to inhibit transcription factors related to innate immunity such as NF-kappa B and interferon regulatory factor-3 (IRF3). In the present study, the effect of ALS-associated optineurin mutations on IRF3 activation was investigated. Optineurin inhibited IRF3 activation induced by melanoma differentiation-associated gene 5 or Toll-IL-1 receptor domain-containing adaptor-inducing interferon-beta. The inhibition was abrogated by mutations related to ALS but not by a mutation related to glaucoma. Reporter assay indicated that the JAK-STAT signaling pathway was not affected by optineurin. These results show that ALS-related optineurin is involved in the IRF3 activation pathway. Pathogenesis of ALS may be associated with some kind of innate immunity, especially that against virus infection, through IRF3 activation.
journal title
Neuroscience Letters
volume
Volume 505
issue
Issue 3
start page
279
end page
281
date of issued
2011
publisher
Elsevier Ireland Ltd
issn
0304-3940
ncid
publisher doi
language
eng
nii type
Journal Article
HU type
Journal Articles
DCMI type
text
format
application/pdf
text version
author
rights
(c) 2011 Elsevier Ireland Ltd. All rights reserved.
relation url
department
Graduate School of Biomedical Science