Huntington’s disease (HD) is a rare hereditary neurodegenerative disorder characterized by multiple metabolic dysfunctions including defects in mitochondrial homeostasis and functions. Although we have recently reported age-related changes in the respiratory capacities in different brain areas in HD mice, the precise mechanisms of how mitochondria become compromised in HD are still poorly understood. In this study, we investigated mRNA and protein levels of selected subunits of electron transport system (ETS) complexes and ATP-synthase in the cortex and striatum of symptomatic R6/2 mice. Our findings reveal a brain-region-specific differential expression of both nuclear and mitochondrial-encoded ETS components, indicating defects of transcription, translation and/or mitochondrial import of mitochondrial ETS components in R6/2 mouse brains.

中文翻译：

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亨廷顿病模型小鼠电子传输系统成分的脑区和细胞室依赖性调节

亨廷顿病 (HD) 是一种罕见的遗传性神经退行性疾病，其特征是多种代谢功能障碍，包括线粒体稳态和功能缺陷。尽管我们最近报道了 HD 小鼠不同大脑区域呼吸能力与年龄相关的变化，但对 HD 中线粒体如何受损的确切机制仍知之甚少。在这项研究中，我们调查了有症状的 R6/2 小鼠的皮层和纹状体中电子传递系统 (ETS) 复合物和 ATP 合酶的选定亚基的 mRNA 和蛋白质水平。我们的研究结果揭示了核和线粒体编码的 ETS 成分的大脑区域特异性差异表达，表明 R6/2 小鼠大脑中线粒体 ETS 成分的转录、翻译和/或线粒体输入存在缺陷。