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首页> 外文期刊>The European Journal of Neuroscience >MRI and in situ hybridization reveal early disturbances in brain size and gene expression in the megencephalic (mceph/mceph) mouse.
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MRI and in situ hybridization reveal early disturbances in brain size and gene expression in the megencephalic (mceph/mceph) mouse.

机译：MRI和原位杂交揭示了中脑型（mceph / mceph）小鼠大脑大小和基因表达的早期紊乱。

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摘要

The mouse model for megencephaly, mceph/mceph, carries a truncating deletion in the Shaker-related voltage gated potassium channel gene 1. Affected mice display neurological disturbances and motor dysfunctions. Symptoms begin to show at 3-4 weeks of age. The cause of the brain enlargement is not clear. To elucidate early events in the development of the disease we used magnetic resonance imaging (MRI) to quantify, over time, the increase in size of several discrete brain regions in the same mice at 3, 8 and 12 weeks of age. We also analysed markers for neuropeptides and growth factors to explore their possible involvement at an early stage. The results show an enlargement of the total coronal area of the brain already at 3 weeks of age. Total brain volume, ventral cortex, hippocampal formation and cerebral cortex were enlarged at 8 weeks and onwards. Thalamus, brainstem, cerebellum and spinal cord did not differ from controls even at 12 weeks. We also report distinct disturbances in the expression of brain-derived neurotrophic factor, insulin-like growth factor binding protein 6 and several neuropeptides at 2 and 3 weeks of age, that is, before an obvious behavioural phenotype can be observed. These results provide an objective description of the size changes in different brain regions of the mceph/mceph mouse, and suggest that certain molecules could be involved in the early processes underlying these changes.
机译：鼠源性脑病的小鼠模型mceph / mceph，在Shaker相关的电压门控钾通道基因1中具有截短的缺失。受影响的小鼠表现出神经系统疾病和运动功能障碍。在3-4周龄时开始出现症状。脑肿大的原因尚不清楚。为了阐明该疾病发展过程中的早期事件，我们使用磁共振成像（MRI）来量化同一只小鼠在3、8和12周龄时几个离散脑区域的大小增加。我们还分析了神经肽和生长因子的标志物，以探讨它们在早期可能参与其中。结果显示，在3周龄时，大脑总冠状区域增大了。在第8周及以后，总脑容量，腹侧皮质，海马形成和脑皮质增大。丘脑，脑干，小脑和脊髓即使在12周时也与对照组没有差异。我们还报告了在2和3周龄时，即在观察到明显的行为表型之前，脑源性神经营养因子，胰岛素样生长因子结合蛋白6和几种神经肽的表达受到明显干扰。这些结果提供了对mceph / mceph小鼠不同大脑区域大小变化的客观描述，并表明某些分子可能参与了这些变化的早期过程。

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