Schiffer D, Autilio-Gambetti L, Chiò A, Gambetti P, Giordana M T, Gullotta F, Migheli A, Vigliani M C
2nd Department of Neurology, University of Turin, Italy.
J Neuropathol Exp Neurol. 1991 Jul;50(4):463-73. doi: 10.1097/00005072-199107000-00007.
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.
包括运动神经元病（MND）在内的几种神经退行性疾病，其特征是形成含有泛素（Ubq）的异常细胞骨架衍生包涵体。我们通过光镜和电镜免疫细胞化学研究了26例MND中Ubq的分布情况。在大多数肌萎缩侧索硬化症（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中未发现。在10 - 15纳米的神经元内细丝中观察到泛素免疫反应性，这些细丝不能被神经丝抗体染色，并且在前角和锥体束的整个神经纤维网中营养不良神经突的致密体上也有观察到。数据分析显示，病程较长或神经元数量较少的病例中，泛素阳性神经元的百分比有降低趋势。在临床病程进展迅速的病例中，泛素阳性包涵体的比例很高，这表明泛素化发生在疾病的早期阶段。
