Mizuno Yuji, Amari Masakuni, Takatama Masamitsu, Aizawa Hitoshi, Mihara Ban, Okamoto Koichi
Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan.
J Neurol Sci. 2006 Nov 1;249(1):13-8. doi: 10.1016/j.jns.2006.05.060. Epub 2006 Jul 3.
An ubiquitin-binding protein, p62, is one of the components of the ubiquitin-containing inclusions in several human neurodegenerative diseases. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of skein-like inclusions, Lewy body-like inclusions, and basophilic inclusions in the remaining anterior horn cells, in which these inclusions contain ubiquitin, while the other characteristic inclusions of Bunina type are ubiquitin-negative. We examined the spinal cord from 28 ALS cases including two ALS with dementia and two ALS with basophilic inclusions, using antibody to p62. The results demonstrated that p62 localized in skein-like inclusions, Lewy body-like inclusions and basophilic inclusions. The number of p62-positive inclusions observed in the remaining anterior horn cells of each section was variable among the ALS cases. In contrast, Bunina bodies, that do not contain ubiquitin, were negative for p62. As far as we examined, the 11 non-ALS cases did not show any p62 immunoreactivities in the anterior horn cells. Our results suggested that p62 plays important roles in forming the inclusions and may be associated with the protection of the neurons from degenerative processes involving ubiquitin.
一种泛素结合蛋白p62，是几种人类神经退行性疾病中含泛素包涵体的组成成分之一。肌萎缩侧索硬化症（ALS）的特征是在剩余的前角细胞中存在丝状包涵体、路易体样包涵体和嗜碱性包涵体，这些包涵体含有泛素，而另一种特征性的布尼纳型包涵体则不含泛素。我们使用p62抗体检测了28例ALS患者的脊髓，其中包括2例合并痴呆的ALS和2例有嗜碱性包涵体的ALS。结果表明，p62定位于丝状包涵体、路易体样包涵体和嗜碱性包涵体中。在各ALS病例中，每个切片剩余前角细胞中观察到的p62阳性包涵体数量各不相同。相比之下，不含泛素的布尼纳小体对p62呈阴性反应。就我们所检测的情况而言，11例非ALS病例在前角细胞中未显示任何p62免疫反应性。我们的结果表明，p62在包涵体形成中起重要作用，并且可能与保护神经元免受涉及泛素的退行性过程有关。
