Fujita Kengo, Ito Hidefumi, Nakano Satoshi, Kinoshita Yoshimi, Wate Reika, Kusaka Hirofumi
Department of Neurology, Kansai Medical University, 10-15, Fumizono-cho, Moriguchi, Osaka 570-8507, Japan.
Acta Neuropathol. 2008 Oct;116(4):439-45. doi: 10.1007/s00401-008-0415-x. Epub 2008 Jul 19.
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.
本报告涉及对成年起病的非典型运动神经元疾病患者嗜碱性包涵体（BIs）中RNA相关蛋白的免疫组织化学研究。对运动皮质和腰脊髓的福尔马林固定、石蜡包埋切片进行了检查。嗜碱性包涵体在苏木精-伊红（H&E）染色和尼氏染色中呈蓝色，在甲基绿-派洛宁染色中呈粉红色。核糖核酸酶预处理消除了甲基绿-派洛宁染色，表明嗜碱性包涵体含有RNA。免疫组织化学显示，嗜碱性包涵体被抗聚腺苷酸结合蛋白1、T细胞胞内抗原1和核糖体蛋白S6的抗体明显标记。这些蛋白是应激颗粒的重要组成成分。相比之下，嗜碱性包涵体对核糖体蛋白L28和去帽酶1无免疫反应，它们分别是转运核糖核蛋白颗粒和加工小体的核心成分。此外，嗜碱性包涵体对TDP-43也无免疫阳性反应。我们的结果表明，翻译衰减可能参与了该疾病中嗜碱性包涵体的形成过程。
