Shashidharan P, Good P F, Hsu A, Perl D P, Brin M F, Olanow C W
Department of Neurology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA.
Brain Res. 2000 Sep 22;877(2):379-81. doi: 10.1016/s0006-8993(00)02702-5.
Parkinson's disease (PD) is a neurodegnerative disorder that is pathologically characterized by the presence of Lewy bodies in the brain. We show that Lewy bodies in PD are strongly immunoreactive for torsinA, the protein product of the DYT1 gene, which is associated with primary generalized dystonia. In the substantia nigra, torsinA immunoreactivity is localized to the periphery of Lewy bodies, whereas, in cortical Lewy bodies it is uniformly distributed. The significance of this finding is unknown, but may implicate torsinA in neuronal dysfunction that occurs in PD as well as in primary dystonia.
帕金森病(PD)是一种神经退行性疾病,其病理特征是大脑中存在路易小体。我们发现,帕金森病中的路易小体对扭转蛋白A具有强烈的免疫反应性,扭转蛋白A是DYT1基因的蛋白质产物,与原发性全身性肌张力障碍有关。在黑质中,扭转蛋白A免疫反应性定位于路易小体的周边,而在皮质路易小体中,它是均匀分布的。这一发现的意义尚不清楚,但可能意味着扭转蛋白A参与了帕金森病以及原发性肌张力障碍中发生的神经元功能障碍。