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首页> 外文期刊>Journal of Molecular Neuroscience: MN >FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathy.
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FUS immunogold labeling TEM analysis of the neuronal cytoplasmic inclusions of neuronal intermediate filament inclusion disease: a frontotemporal lobar degeneration with FUS proteinopathy.

机译：神经元中间细丝包涵体疾病的神经元胞质内含物的FUS免疫金标记TEM分析：FUS蛋白病的额颞叶变性。

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摘要

Fused in sarcoma (FUS)-immunoreactive neuronal and glial inclusions define a novel molecular pathology called FUS proteinopathy. FUS has been shown to be a component of inclusions of familial amyotrophic lateral sclerosis with FUS mutation and three frontotemporal lobar degeneration entities, including neuronal intermediate filament inclusion disease (NIFID). The pathogenic role of FUS is unknown. In addition to FUS, many neuronal cytoplasmic inclusions (NCI) of NIFID contain aggregates of alpha-internexin and neurofilament proteins. Herein, we have shown that: (1) FUS becomes relatively insoluble in NIFID and there are no apparent posttranslational modifications, (2) there are no pathogenic abnormalities in the FUS gene in NIFID, and (3) immunoelectron microscopy demonstrates the fine structural localization of FUS in NIFID which has not previously been described. FUS localized to euchromatin, and strongly with paraspeckles, in nuclei, consistent with its RNA/DNA-binding functions. NCI of varying morphologies were observed. Most frequent were the "loosely aggregated cytoplasmic inclusions," 81% of which had moderate or high levels of FUS immunoreactivity. Much rarer "compact cytoplasmic inclusions" and "tangled twine ball inclusions" were FUS-immunoreactive at their granular peripheries, or heavily FUS-positive throughout, respectively. Thus, FUS may aggregate in the cytoplasm and then admix with neuronal intermediate filament accumulations.
机译：融合于肉瘤（FUS）的免疫反应性神经元和神经胶质内含物定义了一种称为FUS蛋白病的新型分子病理学。 FUS已被证明是具有FUS突变的家族性肌萎缩性侧索硬化的包涵体和三个额颞叶变性实体的组成部分，其中包括神经元中间细丝包涵体病（NIFID）。 FUS的致病作用尚不清楚。除了FUS，NIFID的许多神经元胞质内含物（NCI）还包含α-internexin和神经丝蛋白的聚集体。在本文中，我们表明：（1）FUS在NIFID中变得相对不溶，并且没有明显的翻译后修饰;（2）NIFID中FUS基因中没有致病性异常;（3）免疫电子显微镜显示出良好的结构定位NIFID中FUS的编号，以前没有描述过。 FUS在细胞核中定位于常染色质，并强烈带有副斑点，与其RNA / DNA结合功能一致。观察到各种形态的NCI。最常见的是“松散聚集的细胞质内含物”，其中81％具有中等或高水平的FUS免疫反应性。稀有的“致密细胞质内含物”和“缠结的麻线球内含物”在其颗粒周围呈FUS免疫反应性，或在整个FUS呈阳性。因此，FUS可能在细胞质中聚集，然后与神经元中间细丝堆积物混合。

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