临床试验和脊髓去除在肌萎缩性侧索硬化症的小鼠模型（ALS）
Summary
一个肌萎缩性侧索硬化症（ALS）的小鼠模型研究，临床和行为。作为一个伴随的免疫组织化学分析的前提下编制的脊髓详细描述。
Abstract
肌萎缩性脊髓侧索硬化症（ALS）是一种致命的神经退行性疾病导致运动神经元逐渐退化。零星的疾病，约50多年的家族性疾病的发病高峰是60岁。由于它的前进方向，50％的患者症状发作后30个月内死亡。为了评估这种疾病的新型治疗方案，基于对人类家族中的超氧化物歧化酶基因突变的SOD1（G93A）突变，如ALS的遗传小鼠模型已产生。最重要的方面，必须评估模型是整体存活率，临床过程和运动功能。在这里，我们证明了临床评价，显示两种行为的电机测试的传导，并提供所有参数的定量评分系统。因为在ALS小鼠模型的深入分析，通常需要的脊髓免疫组化检查，我们展示了其应用做准备详细RSAL椎板方法。模范病理结果证实。 ALS小鼠模型的研究中描绘的检查方法的综合应用，将使研究人员能够可靠地测试未来的治疗方案，它可以提供一个为以后的人体临床试验的基础上。
Protocol
动物购自杰克逊实验室（＃002726）1。他们在临床上得分和遭受的运动功能（旋转试验），肌力（悬丝试验）测试。所有这些测试，并以准备脊髓后杀害动物已按照非常接近当地的指引适当进行动物实验。 1。临床评分除了 体重小鼠的评估检查以下4点计分制2电机赤字的迹象： 4分：正常（无运动功能障碍的迹象） …
Discussion
的SOD1（G93A）基因小鼠模型是一个有价值的动物模型，研究病程渐进的运动神经元的损失相比，人类肌萎缩性侧索硬化症8。在这个模型中，各种不同的治疗模式已被评估，并表示以后在人类的临床研究8-10测试的基础上。为了能够检测这些小鼠的实验治疗研究中的显着性差异，这是著名的重要性，包括至少24产仔匹配的性别平衡相同的遗传背景的小鼠和遵循双盲设计8。媲美…
Disclosures
The authors have nothing to disclose.
Acknowledgements
审裁处已收到来自中医药大学哥廷根Forschungsförderungsprogramm的资金支持。 PL和MB分别由东风集团的大脑（郊野公园及海岸公园委员会），哥廷根大学分子生理学研究中心的支持。作者感谢录像和比吉特Liebau的援助，音频和视频编辑的帮助医生拉尔斯Tatenhorst。
Materials
| Manufacturer | Ordering Information | |
| Behavioral testing | ||
| Rota-Rod for Mice | Ugo Basile, Comerio, Italy | # 47600 |
| Hanging wire device | Custom-made | |
| Spinal Cord Preparation | ||
| Operation Table Operation lamp Protective gloves |
||
| “Iris” Scissors, angled to side | Fine Science Tools, Heidelberg, Germany | 14063-09 |
| Cohan-Vannas Spring Scissors, straight | Fine Science Tools, Heidelberg, Germany | 15000-10 |
| Micro forceps | Hammacher, Solingen, Germany | HWC 111-10 |
| Scalpel “präzisa plus” | Dahlhausen, Köln, Germany | 11.000.00.510, FIG 10 |
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Tags
Clinical TestingSpinal Cord RemovalMouse ModelAmyotrophic Lateral SclerosisALSNeurodegenerative DisorderMotoneuronsFamilial MutationsSOD GeneSOD1 (G93A) MutationOverall SurvivalClinical CourseMotor FunctionBehavioural Motor TestsQuantitative Scoring SystemsImmunohistochemical ExaminationSpinal Cord PreparationDorsal Laminectomy MethodHistological FindingsTherapeutic OptionsHuman Clinical Trials
Cite This Article
Günther, R., Suhr, M., Koch, J. C., Bähr, M., Lingor, P., Tönges, L. Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis (ALS). J. Vis. Exp. (61), e3936, doi:10.3791/3936 (2012).