Center for Translational NeuroImaging, Northeastern University Boston, Massachusetts.
Division of Biochemistry, Department of Molecular and Experimental Medicine, The Scripps Research Institute La Jolla, California.
Brain Behav. 2013 Jul;3(4):351-66. doi: 10.1002/brb3.138. Epub 2013 Apr 17.
This study was undertaken to develop a phenotypic model recapitulating the neuropathology of Parkinson's disease (PD). Such a model would show loss of dopamine in the basal ganglia, appearance of Lewy bodies, and the early stages of motor dysfunction. The model was developed by subcutaneously injecting biodegradable microspheres of rotenone, a complex I inhibitor in 8-9 month old, ovariectomized Long-Evans rats. Animals were observed for changes in body weight and motor activity. At the end of 11-12 weeks animals were euthanized and the brains examined for histopathological changes. Rotenone treated animals gain weight and appear normal and healthy as compared to controls but showed modest hypokinesia around 5-6 weeks posttreatment. Animals showed loss of dopaminergic (DA) neurons and the appearance of putative Lewy bodies in the substantia nigra. Neuroinflammation and oxidative stress were evidenced by the appearance of activated microglia, iron precipitates, and 8-oxo-2'-deoxyguanosine a major product of DNA oxidation. The dorsal striatum, the projection site of midbrain DA neurons, showed a significant reduction in tyrosine hydroxylase immunostaining, together with an increase in reactive astrocytes, an early sign of DA nerve terminal damage. Levels of vesicular monoamine transporter 2 (VMAT2) were significantly reduced in the dorsal striatum; however, there was an unexpected increase in dopamine transporter (DAT) levels. Old, ovariectomized females treated with rotenone microspheres present with normal weight gain and good health but a modest hypokinesia. Accompanying this behavioral phenotype are a constellation of neuropathologies characteristic of PD that include loss of DA neurons, microglia activation, oxidative damage to nuclear DNA, iron deposition, and appearance of putative Lewy bodies. This phenotypic model recapitulating the neuropathology of Parkinson's disease could provide insight into early mechanisms of pathogenesis and could aid in the identification of biomarkers to identify patients in early stage, PD.
这项研究旨在开发一种能够重现帕金森病(PD)神经病理学的表型模型。这种模型将表现出基底神经节中多巴胺的丧失、路易体的出现以及运动功能障碍的早期阶段。该模型是通过向 8-9 月龄去卵巢的 Long-Evans 大鼠皮下注射生物可降解的鱼藤酮微球来开发的,鱼藤酮是一种复合体 I 抑制剂。动物被观察体重和运动活动的变化。在 11-12 周结束时,处死动物并检查大脑的组织病理学变化。与对照组相比,鱼藤酮处理的动物体重增加,外观正常且健康,但在治疗后约 5-6 周出现轻微的运动减少。动物表现出多巴胺能(DA)神经元丧失和黑质中潜在路易体的出现。神经炎症和氧化应激的证据是活化的小胶质细胞、铁沉淀和 8-氧-2'-脱氧鸟苷的出现,后者是 DNA 氧化的主要产物。中脑 DA 神经元的投射部位背侧纹状体表现出酪氨酸羟化酶免疫染色的显著减少,同时反应性星形胶质细胞增加,这是 DA 神经末梢损伤的早期迹象。背侧纹状体中囊泡单胺转运体 2(VMAT2)的水平显著降低,但多巴胺转运体(DAT)水平意外增加。接受鱼藤酮微球治疗的老年去卵巢雌性动物体重增加正常,健康状况良好,但运动功能略有减退。伴随这种行为表型的是一系列 PD 特有的神经病理学特征,包括多巴胺能神经元丧失、小胶质细胞活化、核 DNA 氧化损伤、铁沉积和潜在路易体的出现。这种能够重现帕金森病神经病理学的表型模型可以深入了解疾病早期的发病机制,并有助于确定生物标志物以识别早期帕金森病患者。