Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis
Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis
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DOI：
10.1016/j.jns.2010.12.023
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发表时间：
2011-03-15
影响因子：
4.4
通讯作者：
Okamoto, Koichi
中科院分区：
文献类型：
--
作者：
Mizuno, Yuji;Fujita, Yukio;Okamoto, Koichi
外周蛋白是脊髓运动神经元中低表达的III型中间丝蛋白。肌萎缩性侧索硬化症（ALS）的特征是在剩余的前角细胞中存在Bunina小体、skein样包涵体和Lewy小体样包涵体（LBLIs），其中第一和第三结构通过苏木精-伊红（H & E）染色检测到。我们检测了6例ALS患者腰椎脊髓石蜡切片，采用h&e染色和人外周蛋白免疫染色。结果表明，共有73个前角细胞含有一个或多个Bunina小体，其中12个细胞（约16.4%）显示外周蛋白阳性Bunina小体。事实上，尽管在每个神经元中有更多的非免疫反应性Bunina小体，但部分链状Bunina小体表现出外周蛋白阳性反应。LBLIs可见核心对应的外周蛋白免疫染色，但部分LBLIs由于包涵体斜切而表现出不同类型的免疫反应。我们的研究结果表明，尽管外周蛋白在兔体中共定位的机制尚不清楚，但外周蛋白可能参与了这些包涵体的形成。此外，继胱抑素C和转铁蛋白之后，外周蛋白是部分定位于兔体内的第三大流行蛋白。(C) 2010 Elsevier B.V.版权所有
Peripherin is a type III intermediate filament protein expressed with low levels in spinal motor neurons. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of Bunina bodies, skein-like inclusions, and Lewy body-like inclusions (LBLIs) in the remaining anterior horn cells, where the first and third structures are detected by Hematoxylin-Eosin (H & E) staining. We examined paraffin sections of lumbar spinal cords from six ALS patients, using H & E staining and immunostaining for human peripherin. The results demonstrated that there were a total of 73 anterior horn cells containing one or more Bunina bodies, and that twelve of these cells (approximately 16.4%) demonstrated peripherin-positive Bunina bodies. In fact, some part of chain-like Bunina bodies showed peripherin-positive reaction, although there were a much higher number of non-immunoreacitive Bunina bodies in each neuron. LBLIs were clearly immunostained for peripherin corresponding to the core, while some of them showed different types of immunoreactivities due to oblique cutting of inclusions. Our findings suggest that although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions. Furthermore, following cystatin C and transferrin, peripherin is the third most prevalent protein that partially localizes in Bunina bodies. (C) 2010 Elsevier B.V. All rights reserved.