Smith J D, Greenlee J J, Hamir A N, West Greenlee M H
Department of Biomedical Sciences, Iowa State University, Ames, Iowa 50010, USA.
J Comp Pathol. 2008 Jan;138(1):12-22. doi: 10.1016/j.jcpa.2007.09.002. Epub 2007 Dec 3.
Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases characterized microscopically by spongiform lesions (vacuolation) in the neuropil, neuronal loss, and gliosis. Accumulation of the abnormal form of the prion protein (PrP(Sc)) has been demonstrated in the retina of natural and non-natural TSE-affected hosts, with or without evidence of microscopically detectable retinal pathology. This study was conducted to investigate the effect of PrP(Sc) accumulation on retinal neurons in a natural host lacking overt microscopical evidence of retinal degeneration by comparing the distribution of retinal cell type-specific markers in control and scrapie-affected sheep. In retinas with PrP(Sc)-immunoreactivity, there was disruption of the normal immunoreactivity patterns of the alpha isoform of protein kinase C (PKCalpha) and vesicular glutamate transporter 1 (VGLUT1), markers of retinal bipolar cells. Altered immunoreactivity was also observed for microtubule-associated protein 2 (MAP2), a marker of a subset of retinal ganglion cells, and glutamine synthetase (GS), a marker of Müller glia. These results demonstrate alterations of immunoreactivity patterns for proteins associated with specific cell types in retinas with PrP(Sc) accumulation, despite an absence of microscopical evidence of retinal degeneration.
传染性海绵状脑病（TSEs）是一组致命的神经退行性疾病，其微观特征为神经毡中的海绵状病变（空泡化）、神经元丢失和胶质细胞增生。在自然感染和非自然感染TSE的宿主视网膜中，已证实存在异常形式的朊病毒蛋白（PrP(Sc)）积累，无论是否有微观可检测到的视网膜病理证据。本研究旨在通过比较对照羊和羊瘙痒病感染羊视网膜细胞类型特异性标志物的分布，调查在缺乏明显视网膜变性微观证据的自然宿主中，PrP(Sc)积累对视网膜神经元的影响。在具有PrP(Sc)免疫反应性的视网膜中，视网膜双极细胞标志物蛋白激酶Cα（PKCalpha）和囊泡谷氨酸转运体1（VGLUT1）的正常免疫反应模式受到破坏。视网膜神经节细胞亚群标志物微管相关蛋白2（MAP2）和米勒胶质细胞标志物谷氨酰胺合成酶（GS）也观察到免疫反应性改变。这些结果表明，尽管没有视网膜变性的微观证据，但在具有PrP(Sc)积累的视网膜中，与特定细胞类型相关的蛋白质免疫反应模式发生了改变。
