Gai W P, Blessing W W, Blumbergs P C
Department of Physiology, Flinders University of South Australia, Australia.
Brain. 1995 Dec;118 ( Pt 6):1447-59. doi: 10.1093/brain/118.6.1447.
Characteristic ubiquitin-positive, tau-negative, degenerating neurites were present in brainstem regions known to be involved in idiopathic Parkinson's disease. Corresponding changes were entirely absent from controls and from the brainstems obtained from patients who had died with Alzheimer's disease, motor neuron disease and multiple system atrophy. In Parkinson's disease cases degenerating neurites were particularly striking in the dorsal motor nucleus of the vagus. In this nucleus the density of degenerating neurites was inversely related to the duration of Parkinson's disease symptoms. Some ubiquitin-positive degenerating neurites also contained neurofilament immunoreactivity. However, confocal microscopy revealed that ubiquitin and neurofilament reactivities were located in separate regions of the degenerating neurite, suggesting that proteins other than neurofilaments may be important in the process of ubiquitination. The demonstration of ubiquitin-positive degenerating neurites in routinely prepared paraffin-embedded material, particularly in the dorsal motor nucleus of the vagus, could become diagnostically useful in those Parkinson's disease cases in which Lewy bodies are difficult to find. Demonstration of extensive ubiquitin-positive degenerating neurites might provide a clue to disease activity at the time of death.
特征性的泛素阳性、tau蛋白阴性、正在退化的神经突出现在已知与特发性帕金森病相关的脑干区域。对照组以及死于阿尔茨海默病、运动神经元病和多系统萎缩的患者的脑干中完全没有相应变化。在帕金森病病例中,正在退化的神经突在迷走神经背运动核中尤为明显。在这个核中,正在退化的神经突的密度与帕金森病症状的持续时间呈负相关。一些泛素阳性的正在退化的神经突也含有神经丝免疫反应性。然而,共聚焦显微镜显示泛素和神经丝反应性位于正在退化的神经突的不同区域,这表明除神经丝外的其他蛋白质可能在泛素化过程中起重要作用。在常规制备的石蜡包埋材料中,尤其是在迷走神经背运动核中,泛素阳性的正在退化的神经突的显示,在那些难以找到路易小体的帕金森病病例中可能具有诊断价值。广泛的泛素阳性的正在退化的神经突的显示可能为死亡时的疾病活动提供线索。