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首页> 外文期刊>PLoS Genetics >Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis
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Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis

机译：在成年小鼠中消除亨廷顿蛋白可导致进行性行为缺陷，双侧丘脑钙化和脑铁稳态改变

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Huntington’s Disease (HD) is an autosomal dominant progressive neurodegenerative disorder characterized by cognitive, behavioral and motor dysfunctions. HD is caused by a CAG repeat expansion in exon 1 of the HD gene that is translated into an expanded polyglutamine tract in the encoded protein, huntingtin (HTT). While the most significant neuropathology of HD occurs in the striatum, other brain regions are also affected and play an important role in HD pathology. To date there is no cure for HD, and recently strategies aiming at silencing HTT expression have been initiated as possible therapeutics for HD. However, the essential functions of HTT in the adult brain are currently unknown and hence the consequence of sustained suppression of HTT expression is unpredictable and can potentially be deleterious. Using the Cre-loxP system of recombination, we conditionally inactivated the mouse HD gene homologue at 3, 6 and 9 months of age. Here we show that elimination of Htt expression in the adult mouse results in behavioral deficits, progressive neuropathological changes including bilateral thalamic calcification, and altered brain iron homeostasis.
机译：亨廷顿舞蹈病（HD）是一种常染色体显性遗传进行性神经退行性疾病，其特征是认知，行为和运动功能障碍。 HD是由HD基因外显子1中的CAG重复扩增引起的，该基因在编码蛋白亨廷顿蛋白（HTT）中被翻译成扩增的聚谷氨酰胺束。 HD最重要的神经病理学发生在纹状体中，其他大脑区域也受到影响，并在HD病理学中发挥重要作用。迄今为止，尚无HD的治愈方法，近来针对沉默HTT表达的策略已开始作为HD的可能疗法。但是，HTT在成人大脑中的基本功能目前尚不清楚，因此持续抑制HTT表达的结果是不可预测的，并且可能有害。使用Cre-loxP重组系统，我们有条件地灭活了3、6和9个月大的小鼠HD基因同源物。在这里，我们显示消除成年小鼠中的Htt表达会导致行为缺陷，进行性神经病理变化（包括双侧丘脑钙化）和脑铁稳态改变。

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