Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease
Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease
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DOI：
10.1007/s00401-008-0415-x
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发表时间：
2008-10-01
影响因子：
12.7
通讯作者：
Kusaka, Hirofumi
中科院分区：
文献类型：
--
作者：
Fujita, Kengo;Ito, Hidefumi;Kusaka, Hirofumi
本报告涉及对成人发病的非典型运动神经元病患者嗜碱性包涵体 (BI) 中 RNA 相关蛋白的免疫组织化学研究。检查福尔马林固定、石蜡包埋的运动皮层和腰脊髓切片。 BI 在 H&E 和尼氏染色中呈现蓝色，在甲基绿-派洛宁染色中呈现粉红色。核糖核酸酶预处理消除了甲基绿-派洛宁染色，表明 BI 含有 RNA。免疫组织化学显示，BI 明显标记有针对聚腺苷酸结合蛋白 1、T 细胞胞内抗原 1 和核糖体蛋白 S6 的抗体。这些蛋白质是应激颗粒的重要组成部分。相比之下，BI 对核糖体蛋白 L28 和脱帽酶 1 不产生免疫反应，而核糖体蛋白 L28 和脱帽酶 1 分别是转运核糖核蛋白颗粒和加工体的核心成分。此外，BI 的 TDP-43 不呈免疫阳性。我们的结果表明翻译衰减可能参与了这种疾病的 BI 形成过程。
This report concerns an immunohistochemical investigation on RNA-related proteins in the basophilic inclusions (BIs) from patients with adult-onset atypical motor neuron disease. Formalin-fixed, paraffin-embedded sections of the motor cortex and the lumbar spinal cord were examined. The BIs appeared blue in color with H&E and Nissl stain, and pink with methylgreen-pyronin stain. Ribonuclease pretreatment abolished the methylgreen-pyronin staining, suggesting that the BIs contained RNA. Immunohistochemically, the BIs were distinctly labeled with the antibodies against poly(A)-binding protein 1, T cell intracellular antigen 1, and ribosomal protein S6. These proteins are essential constituents of stress granules. In contrast, the BIs were not immunoreactive for ribosomal protein L28 and decapping enzyme 1, which are core components of transport ribonucleoprotein particles and processing bodies, respectively. Moreover, the BIs were not immunopositive for TDP-43. Our results imply that translation attenuation could be involved in the processes of BI formation in this disorder.