Abundant FUS-immunoreactive pathology in the skin of sporadic amyotrophic lateral sclerosis
Abundant FUS-immunoreactive pathology in the skin of sporadic amyotrophic lateral sclerosis
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DOI：
10.1111/ane.12114
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发表时间：
2013-10-01
影响因子：
3.5
通讯作者：
Ono, S.
中科院分区：
文献类型：
--
作者：
Oketa, Y.;Higashida, K.;Ono, S.
目的-肉瘤融合蛋白（FUS）是一种526氨基酸，其表达普遍存在。最近，在家族性肌萎缩性侧索硬化症（ALS）中发现了编码FUS基因的突变。此外，在散发性ALS的神经元胞质包涵体中发现了FUS，这表明FUS在散发性疾病中发生的神经变性中起重要作用。然而，在ALS皮肤中尚无FUS的研究。材料和方法：我们对散发性ALS患者和对照组皮肤中FUS的表达进行了定量免疫组织化学研究。结果-肌萎缩侧索硬化症患者表皮中fus免疫反应性（ir）细胞的比例显著升高(P
Objectives - The fused in sarcoma (FUS) protein is a 526 amino acid and its expression is ubiquitous. Recently, mutations in a gene coding FUS have been identified in familial amyotrophic lateral sclerosis (ALS). Also, FUS has been found in neuronal cytoplasmic inclusions in sporadic forms of ALS, suggesting that FUS has an important role in the neurodegeneration occurring in sporadic disease. However, there has been no study of FUS in ALS skin. Material and methods - We made a quantitative immunohistochemical study of the expression of FUS in the skin from patients with sporadic ALS and controls. Results - The proportion of FUS-immunoreactive (ir) cells in the epidermis in ALS patients was significantly higher (P