Analysis of SOD overexpression mechanism at proteasome hypoactivity condition in FALS

SOD1突变蛋白的积累在FALS的神经元损失机制中起重要作用。错误折叠突变体SOD1蛋白的积累不仅与降解有关，而且与表达水平有关。在转基因小鼠和SOD1突变患者的培养成纤维细胞中，我们发现SOD1在蛋白酶体低活性状态下过表达，p38 MAPK与SOD1过表达有关。我们预计SOD1的过表达在FALS的神经元损失机制中是重要的。

Accumulation of mutant SOD1 protein is important with mechanism of neuronal loss in FALS. Accumulation of missfolding mutant SOD1 protein is associated with not only degradation but also expression level. In cultured fibroblast of transgenic mice and patients with SOD1 mutation, we found SOD1 is overexpressed at proteasome hypoactivity condition and p38 MAPK is associated with SOD1 overexprsssion. We expect this SOD1 overexpression is important with mechanism of neuronal loss in FALS.