Optimal methods to characterize the G93A mouse model of ALS

• Published in: Amyotrophic lateral sclerosis 2005-03, Vol.6 (1), p.55-62
• Main Authors: Miana-Mena, Francisco J., Muñoz, Maria J., Yagüe, Gema, Mendez, Mario, Moreno, Maria, Ciriza, Jesús, Zaragoza, Pilar, Osta, Rosario
• Format: Article
• Language: English
• Subjects: Age Factors; ALS; Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - mortality; Amyotrophic Lateral Sclerosis - physiopathology; Animals; Animals, Newborn; Behavior, Animal; Body Weight - genetics; Disease Models, Animal; electromyography; Electromyography - methods; Evoked Potentials, Motor - physiology; Female; G93A; gender; Genotype; hanging wire test; Male; Mice; Mice, Transgenic; Motor Activity - genetics; Muscle, Skeletal - physiopathology; Psychomotor Performance - physiology; ROC Curve; rotarod test; Sex Factors; Superoxide Dismutase - genetics; Survival Analysis
• ISSN: 1748-2968; 1466-0822; 1471-180X
• DOI: 10.1080/14660820510026162

In the present study, we used the SOD1 (G93A) mutant transgenic mice as a model of amyotrophic lateral sclerosis (ALS). This model is widely used as a laboratory tool to study experimental treatments in vivo for ALS to investigate new therapeutic strategies for this neurodegenerative disease. Such studies require the objective quantification of different parameters while mice develop the disease. We have applied a battery of different and specific tests: scoring of motor deficits by a trained observer, weighing, survival measure, hanging wire test, rotarod task and electromyography, most of them commonly used to evaluate G93A animals. We have critically compared these methods, showing the significant influence of gender on the onset of symptoms, and the optimal moment to apply each test. These results should be taken into account in future therapeutic assays on this ALS model.