Ono S, Takahashi K, Fukuoka Y, Jinnai K, Kanda F, Kurisaki H, Mitake S, Inagaki T, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
J Neurol Sci. 1997 May 29;148(2):193-8. doi: 10.1016/s0022-510x(96)05342-7.
We recently reported a significantly higher incidence of intracytoplasmic inclusion bodies (IIBs) of the substantia nigra in patients with myotonic dystrophy (MyD) than in age-matched controls. The changes are, per se, not specific, since a small percentage of disease and normal controls also showed similar inclusions. To elucidate the pathological significance of the inclusion in MyD, we studied immunohistochemical characteristics of IIBs of the substantia nigra in eight patients with MyD. Many IIBs showed moderately intense immunoreactivity for ubiquitin, microtubule-associated protein (MAP) 1 and MAP 2. However, the IIBs did not react with any of the following: anti-neurofilament protein antibodies (Abs) (68, 160 and 200 kDa), anti-neuron-specific enolase antibody (Ab), anti-tau Ab, anti-tubulin Abs (alpha and beta), anti-paired helical filament Ab, anti-actin Ab, anti-phosphorylated epitope of neurofilaments Ab, anti-synaptophysin Ab, anti-myelin basic protein Ab, anti-actin Ab and anti-glial fibrillary acidic protein Ab. Our results suggest that IIBs of the substantia nigra in MyD are related to an alteration of neuronal cytoskeleton metabolism affecting microtubular proteins in conjunction with activation of ubiquitin proteolytic systems.
我们最近报告称，强直性肌营养不良（MyD）患者黑质的胞质内包涵体（IIBs）发生率显著高于年龄匹配的对照组。这些变化本身并不具有特异性，因为一小部分患病和正常对照也显示出类似的包涵体。为了阐明MyD中包涵体的病理意义，我们研究了8例MyD患者黑质IIBs的免疫组化特征。许多IIBs对泛素、微管相关蛋白（MAP）1和MAP 2显示出中等强度的免疫反应性。然而，IIBs与以下任何一种均无反应：抗神经丝蛋白抗体（Abs）（68、160和200 kDa）、抗神经元特异性烯醇化酶抗体（Ab）、抗tau抗体、抗微管蛋白抗体（α和β）、抗双螺旋丝抗体、抗肌动蛋白抗体、抗神经丝蛋白磷酸化表位抗体、抗突触素抗体、抗髓鞘碱性蛋白抗体、抗肌动蛋白抗体和抗胶质纤维酸性蛋白抗体。我们的结果表明，MyD患者黑质的IIBs与神经元细胞骨架代谢改变有关，这种改变影响微管蛋白，并伴有泛素蛋白水解系统的激活。
