Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA
Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA
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DOI：
10.1016/j.neures.2005.09.006
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发表时间：
2006-01-01
影响因子：
2.9
通讯作者：
Kwak, S
中科院分区：
文献类型：
--
作者：
Kawahara, Y;Sun, H;Kwak, S
Q/R处AMPA受体亚基GluR 2的RNA编辑缺陷。该位点是神经元死亡的主要原因，并且最近已经报道是散发性肌萎缩侧索硬化症（ALS）中运动神经元死亡的紧密相关的病因。我们量化了突变型人铜/锌超氧化物歧化酶（SOD 1）转基因大鼠以及脊髓延髓肌萎缩症（SBMA）患者的单个运动神经元中GluR 2 Q/R位点的RNA编辑效率，发现GluR 2 mRNA在所有检查的运动神经元中完全编辑。在散发性ALS、家族性ALS伴突变型SOD 1和SBMA中，死亡级联反应在垂死的运动神经元中似乎是不同的。(c)2005年Elsevier爱尔兰有限公司和日本神经科学学会。All rights reserved.
Deficient RNA editing of the AMPA receptor subunit GluR2 at the Q/R. site is a primary cause of neuronal death and recently has been reported to be a tightly linked etiological cause of motor neuron death in sporadic amyotrophic lateral sclerosis (ALS). We quantified the RNA editing efficiency of the GluR2 Q/R site in single motor neurons of rats transgenic for mutant human Cu/Zn-superoxide dismutase (SOD1) as well as patients with spinal and bulbar muscular atrophy (SBMA), and found that GluR2 mRNA was completely edited in all the motor neurons examined. It seems likely that the death cascade is different among the dying motor neurons in sporadic ALS, familial ALS with mutant SOD1 and SBMA. (c) 2005 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.