Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1G93A transgenic mice
Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1G93A transgenic mice
复制标题
DOI：
10.1016/j.neurobiolaging.2006.07.014
复制
发表时间：
2007-10-01
影响因子：
4.2
通讯作者：
Cai, Huatibin
中科院分区：
文献类型：
--
作者：
Lin, Xian;Shim, Hoon;Cai, Huatibin
Als2基因的功能障碍与一种形式的青少年起病常染色体隐性遗传性肌萎缩侧索硬化症(ALS)有关。先前的体外研究表明，过表达als2可以保护细胞免受突变型铜锌超氧化物歧化酶(SOD1)诱导的细胞毒性。为了测试als2是否对体内突变的SODI介导的运动神经元变性起到保护作用，我们在als2缺失的背景下研究了SOD1(G93A)小鼠运动神经元疾病的进展。我们的数据表明，als2基因的缺失不影响SODIG93A小鼠的发病。(C)2006 Elsevier Inc.保留所有权利。
Dysfunction of the ALS2 gene has been linked to one form of juvenile onset autosomal recessive amyotrophic lateral sclerosis (ALS). Previous in vitro studies suggest that over-expression of ALS2 protects cells from mutant Cu/Zn superoxide dismutase (SOD1)-induced cytotoxicity. To test whether ALS2 plays a protective role against mutant SODI-mediated motor neuron degeneration in vivo, we examined the progression of motor neuron disease in SOD1 (G93A) mice on an ALS2 null background. Our data suggest that deficiency in the ALS2 gene does not affect the pathogenesis of SOD I G93A mice. (c) 2006 Elsevier Inc. All rights reserved.