4.6 Article

Increased expression of human antiviral protein MxA in FUS proteinopathy in amyotrophic lateral sclerosis

期刊

BRAIN PATHOLOGY
卷 -, 期 -, 页码 -

出版社

WILEY
DOI: 10.1111/bpa.13191

关键词

ALS; FUS; MxA; myxovirus resistance protein; interferon

向作者/读者索取更多资源

FUS mutations are important in familial amyotrophic lateral sclerosis (ALS). FUS-positive neuronal cytoplasmic inclusions (FUS-NCI) are the pathological hallmark, known as FUS proteinopathy. This study examined the expression of MxA in neurons with FUS-NCI and found that increased cytoplasmic MxA expression is associated with the formation of FUS-NCI in ALS-FUS patients.
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.

作者

我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。

评论

主要评分

4.6
评分不足

次要评分

新颖性
-
重要性
-
科学严谨性
-
评价这篇论文

推荐

暂无数据
暂无数据