SOD1-G93A transgenic Mouse Model
SOD1-G93A mice express human SOD1 with the G93A mutation under control of the cistronic human SOD1 promotor. The Super Oxide Dismutase (SOD1) binds free copper and zinc ions and destroys free superoxide radicals in the body. Mutations in this gene have been linked to familial Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig’s disease). The SOD1-G93A mice show a phenotype similar to Amyotrophic Lateral Sclerosis in humans. They develop paralysis in one or more limbs within a few weeks of age.
These mice are a valuable tool to study the influence of new drugs on neuromuscular disorders such as Amyotrophic Lateral Sclerosis.
Figure 1: Longitudinal grip strength measurement of SOD1 mice, lipid peroxidase in the lumbal and thoracal spinal cord and astrocytosis in the ventral thalamus of 18-20 weeks old SOD1 mice. A: Force that animals can use to grip the handle in grams. Two-way ANOVA followed by Bonferroni’s posthoc test. Mean ± SEM; B: TBARS assay, mean + SEM; Two-way ANOVA followed by Bonferroni’s posthoc test. C: Quantitative analysis of astrocytosis (GFAP) in the ventral thalamus. IR: Immunoreactive area; Mean + SEM; t-test; *p<0.05; **p<0.01; ***p<0.001.
QPS Austria also offers research services with the SOD1 low copy number mouse model. Animals develop the same phenotype as the above described SOD1 high copy number mouse model, but disease onset is at about 6-7 months and thus extending the treatment window. Animals survive appr. 6 weeks after symptom onset.
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