LT has received grant support from the Forschungsf&ouml;rderungsprogramm of the University Medicine G&ouml;ttingen. PL and MB were supported by the DFG Research Center for Molecular Physiology of the Brain (CMPB), G&ouml;ttingen. The authors thank Dr. Lars Tatenhorst for assistance with videography and Birgit Liebau for help with audio and video editing.

Animals were purchased from Jackson Laboratory (# 002726) 1. They are clinically scored and subjected to a test of motor function (rotarod test) and of muscular strength (hanging wire test). All these tests and the later killing of the animals in order to prepare the spinal cord have been performed in very close accordance to the local guidelines for proper conduct of animal experiments. 
1. Clinical Score 
Apart from assessment for body weight mice are exa...

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The SOD1 (G93A) genetic mouse model is a valuable animal model to study the disease course of progressive motoneuron loss comparable to human amyotrophic lateral sclerosis 8. A variety of different treatment paradigms have been evaluated in this model and represent a basis for later testing in human clinical studies 8-10. In order to be able to detect significant differences in an experimental treatment study in these mice, it is of eminent importance to include at least 24 litter-matched gender-bal...

<table border="1">
 <tbody>
 <tr>
 <td>&nbsp;</td>
 <td>Manufacturer</td>
 <td>Ordering Information</td>
 </tr>
 <tr>
 <td>Behavioral testing</td>
 <td>&nbsp;</td>
 <td>&nbsp;</td>
 </tr>
 <tr>
 <td>Rota-Rod for Mice</td>
 <td>Ugo Basile, Comerio, Italy</td>
 <td># 47600</td>
 </tr>
 <tr>
 <td>Hanging wire device</td>
 <td>Custom-made</td>
 <td>&nbsp;</td>
 </tr>
 <tr>
 <td colspan="3">&nbsp;</td>
 </tr>
 <tr>
 <td>Spinal Cord Preparation</td>
 <td>&nbsp;</td>
 <td>&nbsp;</td>
 </tr>
 <tr>
 <td>Operation Table Operation lamp Protective gloves</td>
 <td>&nbsp;</td>
 <td>&nbsp;</td>
 </tr>
 <tr>
 <td>&#x201C;Iris&#x201D; Scissors, angled to side</td>
 <td>Fine Science Tools, Heidelberg, Germany</td>
 <td>14063-09</td>
 </tr>
 <tr>
 <td>Cohan-Vannas Spring Scissors, straight</td>
 <td>Fine Science Tools, Heidelberg, Germany</td>
 <td>15000-10</td>
 </tr>
 <tr>
 <td>Micro forceps</td>
 <td>Hammacher, Solingen, Germany</td>
 <td>HWC 111-10</td>
 </tr>
 <tr>
 <td>Scalpel &#x201C;pr&auml;zisa plus&#x201D;</td>
 <td>Dahlhausen, K&ouml;ln, Germany</td>
 <td>11.000.00.510, FIG 10</td>
 </tr>
 </tbody>
</table>

clinical testing and spinal cord removal in a mouse model for amyotrophic lateral sclerosis (als)

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder resulting in progressive degeneration of motoneurons. Peak of onset is around 60 years for the sporadic disease and around 50 years for the familial disease. Due to its progressive course, 50% of the patients die within 30 months of symptom onset. In order to evaluate novel treatment options for this disease, genetic mouse models of ALS have been generated based on human familial mutations in the SOD gene, such as the SOD1 (G93A) mutation. Most important aspects that have to be evaluated in the model are overall survival, clinical course and motor function. Here, we demonstrate the clinical evaluation, show the conduction of two behavioural motor tests and provide quantitative scoring systems for all parameters. Because an in depth analysis of the ALS mouse model usually requires an immunohistochemical examination of the spinal cord, we demonstrate its preparation in detail applying the dorsal laminectomy method. Exemplary histological findings are demonstrated. The comprehensive application of the depicted examination methods in studies on the mouse model of ALS will enable the researcher to reliably test future therapeutic options which can provide a basis for later human clinical trials.

Watch this Scientific Journal Video about Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS at JoVE.com

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS

A mouse model for amyotrophic lateral sclerosis (ALS) is examined clinically and behaviorally. As a prerequisite for an accompanying immunohistological analysis the preparation of the spinal cord is depicted in detail.

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder resulting in progressive degeneration of motoneurons. Peak of onset is around 60 ...

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27.9K vistas.  University Medicine Göttingen. Un modelo de ratón para la esclerosis lateral amiotrófica (ELA) es un examen clínico y de comportamiento. Como un requisito previo para un análisis inmunohistoquímico acompaña la preparación de la médula espinal se representa en detalle.