Quantitative meter assessment in FALS mice: A longitudinal study
Quantitative meter assessment in FALS mice: A longitudinal study
复制标题
DOI：
10.1097/00001756-199709080-00012
复制
发表时间：
1997-09-08
期刊：
影响因子：
1.7
通讯作者：
Moser, P
中科院分区：
文献类型：
--
作者：
Barneoud, P;Lolivier, J;Moser, P
我们在评估运动功能的不同方面的任务中评估了过表达家族性ALS突变SOD 1（Gly-93-->Ala）的转基因小鼠的G1 H系，以确定这些缺陷可以多早被检测到以及它们的出现顺序。最早的赤字中观察到的肌肉力量和协调测试早在8周龄和他们的发展似乎是双相的，而自发活动不受损，直到15周龄。这些研究表明，除了先前证明的组织学和肌电图缺陷，这种转基因小鼠还表现出运动功能的变化，使人联想到人类疾病，加强和扩展其作为家族性肌萎缩性侧索硬化症（ALS）动物模型的有效性，并允许研究ALS的新型药物治疗。
WE have evaluated the G1H line of transgenic mice overexpressing a familial ALS mutation of SOD1 (Gly-93-->Ala) in tasks assessing different aspects of motor function to determine how early these deficits could be detected and their order of appearance. The earliest deficits were observed in tests of muscle strength and coordination as early as 8 weeks of age and their development appeared to be biphasic, whereas spontaneous activity was not impaired until 15 weeks of age. These studies show that, in addition to the previously demonstrated histological and electromyographic deficits, this transgenic mouse also presents changes in motor function reminiscent of the human disease, reinforcing and extending its validity as an animal model of familial amyotrophic lateral sclerosis (FALS) and allowing the investigation of novel drug treatment for ALS.