Duchenne muscular dystrophy (DMD) is a progressive muscular disorder caused by an imbalance between muscle degeneration and regeneration resulting in muscle degeneration, necrosis, accumulation of fat and fibrosis, and insufficient regeneration/loss of myofibers. The genetic cause of DMD are mutations of the dystrophin muscular dystrophy gene (DMD) on the X chromosome. The Dmdmdx mutation in mice has a termination codon in exon 23 that is predicted to result in a truncated protein. Homozygous females and hemizygous males develop the myopathic features of DMD; although the myopathology is both less severe than the human disease course.


The Tg(Myh6-DMD*)47Dua transgene carries a deletion of repeat 4 through repeat 23 and the C-terminus under the control of the rat cardiac muscle specific alpha myosin (Myh6 or &#945-MHC) promoter. When the transgene is combined with the mdx allele, the donating investigator reports that homozygous mice exhibit partially improved cardiac muscle function and histopathology as compared to mdx mice.

Mice homozygous/hemizygous for the spontaneous Dmdmdx allele and homozygous for the human micro-dystrophin transgene exhibit partially improved cardiac muscle function and muscle histopathology as compared to mdx mice and may be useful for studying Duchenne muscular dystrophy.

This strain is hosted by NIH's MMRRC, which partners with JAX for the distribution of this and other strains. For additional information and to purchase, please visit the MMRRC site.