Developed by Dr. Takeda’s group at NCNP, Japan, the MDX-23 mutation does not disrupt the expression of four other shorter isoforms that are also expressed from the dystrophin gene through differential promoter usage. The skeletal muscles are hypertrophic, and the muscles exhibit variations in fiber size, with evidence of necrosis and regeneration. C57BL/6 mice are the wild-type controls for this model.
Hathout, Y., Marathi, R. L., Rayavarapu, S., Zhang, A., Brown, K. J., Seol, H., … McDonald, C. (2014). Discovery of serum protein biomarkers in the mdx mouse model and cross-species comparison to Duchenne muscular dystrophy patients. Human Molecular Genetics, 23(24), 6458–6469. Publication
Rayavarapu, S., Coley, W., Cakir, E., Jahnke, V., Takeda, S., Aoki, Y., … Nagaraju, K. (2013). Identification of Disease Specific Pathways Using in Vivo SILAC Proteomics in Dystrophin Deficient mdx Mouse. Molecular & Cellular Proteomics : MCP, 12(5), 1061–1073. Publication
Aoki, Y., Yokota, T., Nagata, T., Nakamura, A., Tanihata, J., Saito, T., … Takeda, S. (2012). Bodywide skipping of exons 45–55 in dystrophic mdx52 mice by systemic antisense delivery. Proceedings of the National Academy of Sciences of the United States of America, 109(34), 13763–13768. Publication
Echigoya, Y., Aoki, Y., Miskew, B., Panesar, D., Touznik, A., Nagata, T., … Yokota, T. (2015). Long-Term Efficacy of Systemic Multiexon Skipping Targeting Dystrophin Exons 45–55 With a Cocktail of Vivo-Morpholinos in Mdx52 Mice. Molecular Therapy. Nucleic Acids, 4(2), e225–. Publication