Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis
Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis
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DOI：
10.1007/s10529-007-9595-z
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发表时间：
2008-04-01
影响因子：
2.7
通讯作者：
Costa, Julia
中科院分区：
文献类型：
--
作者：
Gomes, Catarina;Palma, Angelina S.;Costa, Julia
肌萎缩侧索硬化症（ALS）运动神经元的高尔基体（GA）出现破坏。在此，构建了稳定表达人超氧化物歧化酶1（hSOD 1）（wt）和突变体hSOD 1（G93 A）的小鼠运动神经元样NSC-34细胞系作为ALS细胞模型。GA破坏的细胞数量从14%增加到34%。此外，NSC-34/hSOD 1（G93 A）细胞显示较低水平的增殖和分化。GA中断不是由细胞凋亡引起的，如通过包括半胱天冬酶-3激活的几种技术所确定的。类似地，ALS患者的脊髓没有显示caspase-3激活。因此，NSC-34/hSOD 1（G93 A）细胞是研究ALS中GA功能障碍的合适细胞模型。
The Golgi apparatus (GA) appears disrupted in motor neurons of amyotrophic lateral sclerosis (ALS). Here, mouse motor neuron-like NSC-34 cell lines stably expressing human superoxide dismutase 1 (hSOD1)(wt) and mutant hSOD1(G93A), as an ALS cell model, were constructed. The number of cells with disrupted GA increased from 14% to 34%. Furthermore, NSC-34/hSOD1(G93A) cells showed lower levels of proliferation and differentiation. GA disruption was not caused by apoptosis as determined by several techniques including caspase-3 activation. Similarly, spinal cords from ALS patients did not show caspase-3 activation. Therefore, NSC-34/hSOD1(G93A) cells are a suitable cell model to study GA dysfunction in ALS.