Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis
Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis
复制标题
DOI：
10.1016/j.nmd.2005.02.005
复制
发表时间：
2005-05-01
影响因子：
2.8
通讯作者：
Cheema, SS
中科院分区：
文献类型：
--
作者：
Atkin, JD;Scott, RL;Cheema, SS
进行这项研究的目的是确定与野生型小鼠相比，SOD1 G93A 小鼠的代表性慢肌和快肌的症状前或末期患病肌纤维的组织学、病理学和收缩特性是否发生改变。在出现症状前的 SOD1 G93A 小鼠中，没有检测到外周功能障碍，这提供了肌肉病理继发于运动神经元功能障碍的证据。然而，在疾病末期，单肌纤维收缩分析表明，快肌纤维和神经肌肉接头优先受到肌萎缩侧索硬化症引起的去神经支配的影响，在被钙激活时无法产生与年龄匹配对照的肌纤维相同水平的力。还检查了这些肌肉中转基因 SOD1 表达、聚集状态和活性的水平，但对肌纤维类型没有偏好。因此，SOD1 G93A 小鼠中 SOD1 蛋白表达/活性与肌纤维类型脆弱性之间不存在简单的相关性。 © 2005 Elsevier B.V. 保留所有权利。
This investigation was undertaken to determine if there are altered histological, pathological and contractile properties in presymptomatic or endstage diseased muscle fibres from representative slow-twitch and fast-twitch muscles of SOD1 G93A mice in comparison to wildtype mice. In presymptomatic SOD1 G93A mice, there was no detectable peripheral dysfunction, providing evidence that muscle pathology is secondary to motor neuronal dysfunction. At disease endstage however, single muscle fibre contractile analysis demonstrated that fast-twitch muscle fibres and neuromuscular junctions are preferentially affected by amyotrophic lateral sclerosis-induced denervation, being unable to produce the same levels of force when activated by calcium as muscle fibres from their age-matched controls. The levels of transgenic SOD1 expression, aggregation state and activity were also examined in these muscles but there no was no preference for muscle fibre type. Hence, there is no simple correlation between SOD1 protein expression/activity, and muscle fibre type vulnerability in SOD1 G93A mice. © 2005 Elsevier B.V. All rights reserved.