Departments of Neuropathology Neuroanatomy, Cell Biology and Histology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
Neuropathol Appl Neurobiol. 2012 Jun;38(4):322-8. doi: 10.1111/j.1365-2990.2011.01217.x.
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.
We immunohistochemically examined the brain, spinal cord and peripheral ganglia from five patients with INIBD and five control subjects, using anti-FUS antibodies.
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.
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.
最近的研究表明，肉瘤融合蛋白（FUS）是核内包涵体病（INIBD）患者大脑中“神经元”核内包涵体（INIBs）的组成部分。然而，FUS 免疫反应结构在 INIBD 中的程度和频率尚不确定。
我们使用抗 FUS 抗体对 5 例 INIBD 患者和 5 例对照的脑、脊髓和周围神经节进行了免疫组织化学检查。
在对照组中，神经元和神经胶质细胞的核均强烈标记有抗 FUS，神经元细胞质对 FUS 呈弱阳性。在 INIBD 中，脑和脊髓中的神经元和神经胶质 INIB 呈 FUS 阳性。FUS 阳性 INIB 也存在于周围神经节中。FUS 阳性神经元 INIB 相对于含包涵体神经元总数的比例为 55.6%至 83.3%（平均 73.2%），FUS 阳性神经胶质 INIB 比例为 45.9%至 85.7%（平均 62.7%）。含包涵体神经元和神经胶质细胞的核和细胞质均无 FUS 免疫反应性。
这些发现表明 FUS 被纳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表达减少和/或包涵体内的隔离。
