Generation of disease models for neurodegenerative disorders by deleting selenoprotein synthesis

我们通过删除抗氧化酶的主要成员硒蛋白来建立神经退行性疾病的疾病模型。我们发现转录因子Nrf2在硒蛋白缺乏的神经元组织中被激活。在缺乏Nrf2的情况下，神经元组织中的硒蛋白缺乏会导致严重的退行性表型。这些结果表明，硒蛋白对维持神经元组织的动态平衡是必不可少的，而Nrf2对硒蛋白的丢失具有细胞保护作用。

We generated disease models for neurodegenerative disorders by deleting selenoproteins, a major member of antioxidant enzymes. We have found that transcriptional factor Nrf2 activate in the selenoprotein-deficient neuronal tissue. In the absence of Nrf2, selenoprotein deficiency in neuronal tissue cause severe degenerative phenotype. These results reveal that selenoproteins are essential for maintaining homeostasis of neuronal tissue, and that Nrf2 has cytoprotective role against loss of selenorpoteins.