Duchenne Muscular Dystrophy – DMD Mouse Model (mdx mouse)
Perform behavioral research studies to investigate your novel and innovative targeted Duchenne Muscular Dystrophy therapies using the DMD mouse modelÂ
DMD Mouse Model Key Characteristics:
Duchenne muscular dystrophy (DMD) is a neuromuscular disorder characterized by progressive skeletal muscle weakening and wasting. To test the efficacy of novel therapies or disease-modifying treatments for DMD, multiple research models can be used. Out of these, the most commonly used is the mdx (B10. mdx) mouse model. The mdx mouse model closely recapitulates the human DMD pathophysiological characteristics due to an X-linked mutation in the gene that encodes dystrophin in the skeletal muscle membrane.Â
Mdx mice show progressive cycles of skeletal muscle degeneration and regeneration that mimic human course of DMD, starting at approximately three weeks of age. As part of its preclinical research services, InnoSer performs efficacy studies in the mdx mouse model, focusing on functional readouts (skeletal muscle strength, motor function) as well as histopathology analyses (e.g., WGA, Sirius red, H&E) of skeletal muscle (regeneration, necrosis, hypertrophy).Â
✓ The mdx mutation results in a termination codon of the dystrophin muscular dystrophy (Dmd) gene on the X chromosome.
✓ Mdx mice show robust muscle function impairments at a young age and display cognitive impairments under specific conditions (Remmelink et al. 2016; Engelbeen et al. 2021).Â
Take advantage of InnoSer’s expertise, flexibility, and collaborative approach for your research. Our in-house neurology experts have long-standing experience with performing preclinical behavioural research services in in vivo models and help guide your decision on choosing the best model fit for your current research goals.Â
InnoSer’s neurology expert team possesses relevant experience in working with multiple therapy types ranging from small molecules, peptides, enzymes, oligonucleotides, gene therapy (viral vectors – e.g.. AAVs) and immunotherapies (antibody/vaccine immunotherapies).Â
Your Neurology Research Starts Here.
Choose the Right Model for Your Research with Confidence
DMD Mouse Model Sample Data
Histopathology images show differences between the m. tibialis anterior skeletal muscle of wild-type and DMD mouse.
Compared to WT (A), DMD (B) mice show centrally located nuclei (DAPI, blue). Centrally located nuclei are hallmark of skeletal muscle regeneration, and in this model, reflective of the constant remodeling of the skeletal muscle driven by cycles of muscle degeneration and regeneration. In WT mice, nuclei are located at the sarcolemma (WGA, green) of the skeletal muscle fibers.Â
Body weight progression over time
MDX mice (B10.mdx) show normal body weight progression over span of 7 weeks which is comparable to healthy controls.
Four limb hanging test
MDX mice (B10.Mdx) perform significantly worse in ​the four-limb hanging test compared to healthy WT controls.
The C57BL/10ScSnJ mdx mouse model for Duchenne Muscular Dystrophy shows significant muscle strength impairment
C57BL/10ScSnJ mdx mice show significant decreases in physical performance in the rotarod test in comparison to their WT litter mates at 4 weeks and 7 weeks of age (**P<0.001). The Rotarod is the golden standard for assessing motor performance and learning in mice. The mice are placed on a rotating rod, with increasing rotating speed. Motor performance is measured by the maximal RPM (rounds per minute) at which mice are able to keep up with the rotating rod. Motor learning can be assessed by training mice on the rod for several trials.Â
DMD Mouse Model Readouts
Biological Readouts
Test the efficacy of your treatments with the following biological readouts:Â
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- Histopathology (e.g., WGA, Sirius red, H&E etc.,)Â of skeletal muscle (regeneration, necrosis, hypertrophy) and heart tissue.
Our Team’s Featured Publications
- Remmelink, E., Aartsma-Rus, A., Smit, A. B., Verhage, M., Loos, M., & van Putten, M. (2016). Cognitive flexibility deficits in a mouse model for the absence of full-length dystrophin. Genes, brain, and behavior, 15(6), 558–567. https://doi.org/10.1111/gbb.12301Â
- Engelbeen, S., Aartsma-Rus, A., Koopmans, B., Loos, M., & van Putten, M. (2021). Assessment of Behavioral Characteristics With Procedures of Minimal Human Interference in the mdx Mouse Model for Duchenne Muscular Dystrophy. Frontiers in behavioral neuroscience, 14, 629043. https://doi.org/10.3389/fnbeh.2020.629043Â
The People Behind Your Research
Thomas Vogels, PhD, In Vivo Neurology Study Director
Leads an expert team of scientists with vast experience in our Neurology models to help you choose the right model and guide your optimal study design. We provide the solution to accelerating your drug development.
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