Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis
Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis
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DOI：
10.1016/j.neulet.2003.07.010
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发表时间：
2003-11-20
影响因子：
2.5
通讯作者：
Zona, C
中科院分区：
文献类型：
--
作者：
Pieri, M;Albo, F;Zona, C
各种证据表明，肌萎缩侧索硬化症（ALS）选择性地影响运动神经元的功能，但ALS中单个运动神经元的电生理改变仍有待记录。在目前的工作中，运动神经元的兴奋性已经在ALS的家族形式的转基因小鼠模型中进行了测试，与Cu，Zn超氧化物歧化酶（Gly（93）--> Ala）的突变相关。膜片钳记录转基因小鼠运动神经元的膜电位显示，与对照小鼠的运动神经元相比，它们的放电频率增加，持续时间缩短。然而，这些神经元的被动膜特性是等同的。这些结果表明，运动神经元兴奋性的改变伴随着ALS相关的突变，并可能有助于疾病的发病机制。(C)2003爱思唯尔爱尔兰有限公司保留所有权利。
Various evidence suggests that amyotrophic lateral sclerosis (ALS) selectively affects motor neuron functioning, but electrophysiological alterations of single motor neurons in ALS remains to be documented. In the present work, the excitability of motor neurons has been tested in a transgenic mouse model of a familial form of ALS, associated with a mutation in Cu,Zn superoxide dismutase (Gly(93) --> Ala). Patch-clamp recordings of membrane potential in transgenic mice motor neurons showed that they fire with increased frequency and shorter duration compared to motor neurons from control mice. The passive membrane properties of these neurons were equivalent however. Such results suggest that an altered motor neuron excitability accompanies an ALS associated mutation and that may contribute to the pathogenesis of the disease. (C) 2003 Elsevier Ireland Ltd. All rights reserved.