The MitoPark mouse--a new model for Parkinson's Disease

我们使用反向遗传方法来研究这个问题，并创建了中脑DA神经元mtDNA表达降低的条件敲除小鼠。这些小鼠有缓慢进展的成年发作，典型的帕金森症状，伴有细胞内包涵体的进行性形成和特征性神经病理。包裹体存在于大多数DA神经元中，包含线粒体膜和蛋白质。因此，我们的研究结果支持了呼吸链功能障碍帕金森病的病因学作用，并提出了路易小体形成的新机制。

We have used a reverse genetic approach to investigate this question and created conditional knockout mice with reduced mtDNA expression in midbrain DA neurons. These mice have adult onset of slowly progressive, typical parkinsonian symptoms accompanied by progressive formation of intracellular inclusions and characteristic neuropathology. The inclusions are present in most DA neurons and contain both mitochondrial membranes and proteins. Our findings thus support an etiological role for respiratory chain dysfunction Parkinsons disease and suggest a novel mechanism for the formation of Lewy bodies.