Duchenne Muscular Dystrophy (DMD) Mouse Model
Perform behavioural research studies to investigate your novel and innovative targeted Duchenne Muscular Dystrophy therapies using the mdx mouse model
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.
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).
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.
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).
Mdx mouse key model characteristics:
- 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).
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Key readouts in the DMD mouse model
Test the efficacy of your treatments with the following commonly used behavioral tests:
Motor function:
- Grip Strength Test
- CatWalk™ Automated Gait Analysis System
- Rotarod
- Wire Hanging
- Four limb wire hanging test
- Functional reach test
- Treadmill
Assess the efficacy of your treatments with the following biological readouts:
- Histopathology (e.g., WGA, Sirius red, H&E etc.,) of skeletal muscle (regeneration, necrosis, hypertrophy) and heart tissue.
Example data featuring the DMD mouse model:
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.
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