Ubiquitin in Motor Neuron Disease: Study at the Light and Electron Microscope
Ubiquitin in Motor Neuron Disease: Study at the Light and Electron Microscope
复制标题
运动神经元疾病中的泛素：光学和电子显微镜研究
DOI：
10.1097/00005072-199107000-00007
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发表时间：
1991
影响因子：
3.2
通讯作者：
M. Vigliani
中科院分区：
文献类型：
--
作者：
D. Schiffer;L. Autilio‐Gambetti;A. Chiò;P. Gambetti;F. Gullotta;A. Migheli;M. Vigliani
包括运动神经元病（MND）在内的几种神经退行性疾病的特征在于形成含有泛素（Ubq）的异常细胞色素衍生的包涵体。本文用光镜和电镜免疫细胞化学方法研究了26例MND中Ubq的分布。在大多数肌萎缩侧索硬化（ALS）病例的前角神经元中发现了泛素阳性包涵体，但在其他形式的MND中不存在。在10-15 nm的神经元内细丝中观察到泛素免疫反应性，这些细丝未被神经丝抗体染色，并且在整个前角和锥体束的神经突起中的营养不良神经突的致密体上观察到泛素免疫反应性。数据分析显示，在病程较长或神经元数量较少的病例中，Ubq阳性神经元的百分比有降低的趋势。在具有侵袭性临床病程的病例中，Ubq阳性内含物的比例很高，这表明遍在蛋白化发生在疾病的早期阶段。
Several neurodegenerative diseases, including motor neuron disease (MND), are characterized by formation of abnormal cytoskeleton-derived inclusions which contain ubiquitin (Ubq). We have studied the distribution of Ubq in 26 cases of MND with light and electron microscopic immunocytochemistry. Ubiquitin-positive inclusions were found in neurons of anterior horns in most cases of amyotrophic lateral sclerosis (ALS) but were not present in other forms of MND. Ubiquitin immunoreactivity was observed in 10–15 nm intraneuronal filaments, which were not stained by antibodies to neurofilaments, and on dense bodies of dystrophic neurites throughout the neuropil of anterior horns and pyramidal tracts. Data analysis showed a trend toward lower percentage of Ubq-positive neurons in cases with longer duration of illness or lower number of neurons. A high percentage of Ubq-positive inclusions occurred in cases with an aggressive clinical course, suggesting that ubiquitination takes place at early stages of the disease.