Altered macroautophagy in the spinal cord of S0D1 mutant mice
Altered macroautophagy in the spinal cord of S0D1 mutant mice
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DOI：
10.4161/auto.5524
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发表时间：
2008-04-01
期刊：
影响因子：
13.3
通讯作者：
Le, Weidong
中科院分区：
文献类型：
--
作者：
Li, Liang;Zhang, Xiaojie;Le, Weidong
肌萎缩侧索硬化症(ALS)是一种选择性运动神经元丢失(MNS)引起的神经退行性疾病。约20%的家族性ALS(FALS)患者携带铜锌超氧化物歧化酶(SOD1)基因突变，该基因突变在ALS的发病机制中起重要作用。有证据表明，宏自噬在体外可以降解突变的SOD1。为了探讨突变型SOD1是否能在体内诱导巨噬细胞，我们检测了不同时期SOD1(G93A)转基因小鼠脊髓中的LC3处理过程和巨噬细胞的激活状态。我们的数据表明SOD1(G93A)小鼠的脊髓中自噬被激活，这表明巨自噬可能在ALS的发病机制中起作用。
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by selective loss of motor neurons (MNs). About 20% familial cases of ALS (fALS) carried the Cu, Zn-superoxide dismutase (SOD1) gene mutation, which plays a crucial role in the pathogenesis of fALS. There is evidence suggesting that macro-autophagy can degrade mutated SOD1 in vitro. To investigate whether the mutant SOD1 can induce macroautophagy in vivo, we examined the LC3 processing in spinal cord and the activation status of macroautophagy in MNs of SOD1(G93A) transgenic mice at different stages. Our data demonstrated that autophagy was activated in spinal cord of SOD1(G93A) Mice indicating a possible role of macroautophagy in the pathogenesis of ALS.