Mizuno Yuji, Ohama Eisaku, Hirato Junko, Nakazato Yoichi, Takahashi Hitoshi, Takatama Masamitsu, Takeuchi Toshiyuki, 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 Jul 15;246(1-2):131-7. doi: 10.1016/j.jns.2006.02.019. Epub 2006 Apr 17.
Nestin, an intermediate filament protein, is mainly expressed in neural progenitor/stem cells in the central nervous system. Recently, we reported that nestin is expressed in Purkinje cells in patients with Creutzfeldt-Jakob disease (CJD). In this study, we examined a total of 19 CJD cerebella to analyze the intensity and pattern of nestin immunoreactivity of Purkinje cells in different pathological stages of degeneration in the cerebellar cortex. The results showed that the Purkinje cells were immunoreactive with nestin regardless of the severity of degenerative cerebellar cortex. Furthermore, we noted several different types of nestin immunoreactivity, indicated by diffuse and fine, coarse, and inclusion-like immunostainings within Purkinje cell bodies as well as dot-like staining outside of the cell bodies. In contrast, on examination of cerebella from non-CJD patients, 6 of 30 cases showed nestin immunoreactivity to a lesser extent. Thus, nestin-positive Purkinje cells are more common in CJD cerebella than in non-CJD cerebella. Although the mechanism of nestin expression in Purkinje cells is not yet understood, we suggest that such nestin-positive Purkinje cells are being reactivated to survive the cell death.
巢蛋白是一种中间丝蛋白，主要表达于中枢神经系统的神经祖细胞/干细胞中。最近，我们报道了巢蛋白在克雅氏病（CJD）患者的浦肯野细胞中表达。在本研究中，我们共检查了19例CJD患者的小脑，以分析小脑皮质不同病理退变阶段浦肯野细胞巢蛋白免疫反应的强度和模式。结果显示，无论小脑皮质退变的严重程度如何，浦肯野细胞均对巢蛋白呈免疫反应。此外，我们注意到几种不同类型的巢蛋白免疫反应，表现为浦肯野细胞体内弥漫性、细微、粗大及包涵体样免疫染色，以及细胞体外的点状染色。相比之下，在检查非CJD患者的小脑时，30例中有6例显示出程度较轻的巢蛋白免疫反应。因此，巢蛋白阳性的浦肯野细胞在CJD患者的小脑中比在非CJD患者的小脑中更常见。虽然浦肯野细胞中巢蛋白表达的机制尚不清楚，但我们认为这种巢蛋白阳性的浦肯野细胞正在被重新激活以在细胞死亡中存活。
