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首页> 外文期刊>Neuropathology and applied neurobiology >FUS immunoreactivity of neuronal and glial intranuclear inclusions in intranuclear inclusion body disease
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FUS immunoreactivity of neuronal and glial intranuclear inclusions in intranuclear inclusion body disease

机译：神经元和神经胶质核内包裹体在核内包涵体疾病中的FUS免疫反应性

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Aims: Recent studies have shown that fused-in-sarcoma (FUS) protein is a component of 'neuronal' intranuclear inclusion bodies (INIBs) in the brains of patients with intranuclear inclusion body disease (INIBD). However, the extent and frequency of FUS-immunoreactive structures in INIBD are uncertain. Methods: We immunohistochemically examined the brain, spinal cord and peripheral ganglia from five patients with INIBD and five control subjects, using anti-FUS antibodies. Results: In controls, the nuclei of both neurones and glial cells were intensely immunolabelled with anti-FUS and neuronal cytoplasm was weakly positive for FUS. In INIBD, neuronal and glial INIBs in the brain and spinal cord were positive for FUS. FUS-positive INIBs were also found in the peripheral ganglia. The proportion of FUS-positive neuronal INIBs relative to the total number of inclusion-bearing neurones ranged from 55.6% to 83.3% (average 73.2%) and that of FUS-positive glial INIBs ranged from 45.9% to 85.7% (average 62.7%). The nucleus and cytoplasm of inclusion-bearing neurones and glial cells showed no FUS immunoreactivity. Conclusions: These findings suggest that FUS is incorporated into INIBs in both neurones and glial cells and that loss of normal FUS immunoreactivity may result from reduced protein expression and/or sequestration within inclusions.
机译：目的：最近的研究表明，融合肉瘤（FUS）蛋白是核内包涵体病（INIBD）患者大脑中“神经元”核内包涵体（INIB）的组成部分。然而，INIBD中FUS免疫反应结构的范围和频率尚不确定。方法：我们使用抗FUS抗体对5例INIBD患者和5例对照受试者的脑，脊髓和周围神经节进行了免疫组织化学检查。结果：在对照组中，神经元和神经胶质细胞的核均被抗FUS强烈免疫标记，而神经元细胞质对FUS呈弱阳性。在INIBD中，大脑和脊髓的神经元和神经胶质INIB对FUS呈阳性。在外周神经节中也发现了FUS阳性的INIB。 FUS阳性神经元INIB相对于带有包涵体的神经元总数的比例为55.6％至83.3％（平均73.2％），而FUS阳性神经胶质INIB的比例为45.9％至85.7％（平均62.7％）。 。带有包涵体的神经元和神经胶质细胞的细胞核和细胞质均未显示FUS免疫反应性。结论：这些发现表明，FUS被掺入神经元和神经胶质细胞的INIBs中，正常FUS免疫反应性的丧失可能是由于蛋白质表达的降低和/或内含物的螯合。

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