Muscular Dystrophy Mouse Model Resource

The Jackson Laboratory has a large collection of mouse models of muscular dystrophy. They are categorized below by specific disease type to help you easily find the best strain for your research:

See a complete list of JAX® Mice for Muscular Dystrophy research

Comparison of featured mouse models of Duchenne Muscular Dystrophy

This table summarizes the differences among important mouse models for Duchenne Muscular Dystrophy to help you easily find the right strain for your research:

Strain Name Common Name Molecular Mutation Phenotype Survival

C57BL/10ScSn-Dmdmdx/J

001801

 mdx Spontaneous C-to-T transition at position 3185 (exon 23) resulting in truncated protein Skeletal muscle fibers undergo cycles of degeneration and regeneration beginning at 3 weeks, necrosis by 9 weeks, increased satellite cell proliferation, abnormal grip strength and impaired coordination Greater than 1.5 years

B6Ros.Cg-Dmdmdx-4Cv/J

002378

 mdx4cv ENU induced C-to-T transition at position 7916 (exon 53) resulting in premature stop codon Skeletal muscle fibers undergo cycles of degeneration and regeneration (although fewer revertants than other strains) accompanied by necrosis, fibrosis and centrally located nuclei, abnormal muscle contractility and grip strength Not determined

STOCK Utrntm1Ked

Dmdmdx/J

014563

 mdx-
uturn-/-		 Null targeted mutation in Utrn and a spontaneous C-to-T transition at position 3185 (exon 23) resulting in truncated DMD protein Abnormal muscle fiber morphology and size, rapid and progressive loss of body weight, reduced mobility, waddling gate, muscle weakness and kyphosis by 4-6 weeks of age Premature death by 20 weeks

Featured JAX® Mice Models of Muscular Dystrophy

C57BL/10ScSn-Dmdmdx/J
001801

  • Common name: mdx
  • Genetic background: C57BL/10ScSn
  • Spontaneous C-to-T transition at position 3185 (exon 23) in the dystrophin muscular dystrophy (Dmd) gene, resulting in a termination codon in place of a glutamine codon, and expression of a truncated protein (Bulfield et al. 1984, Sicinski 1989)
  • Progressive muscle degeneration and atrophy beginning at 3 weeks of age, skeletal muscle necrosis at 9 weeks of age
  • Consistent with X-inactivation, heterozygous females have a mosaic absence of dystrophin, but this diminishes with age such that at 10 days of age 37% of the fibers in a cross section of the quadriceps lack dystrophin staining, this diminishes to 18% at 35 days and 4% by 60 days of age
B6Ros.Cg-Dmdmdx-4Cv/J
002378

  • Common name: mdx4cv
  • Genetic background: C57BL/6Ros; fully congenic
  • Chemically-induced (ENU) C-to-T transition at position 7916 (exon 53) in the dystrophin muscular dystrophy (Dmd) gene, resulting in a premature termination codon (Chapman et al. 1989, Im et al. 1996)
  • Progressive muscle fiber necrosis, fibrosis and centrally nucleated skeletal muscle fibers
  • Fewer muscle fiber revertants observed in quadricep cross-sections than in mice with Dmdmdx, Dmdmdx-2Cv and Dmdmdx-3Cv mutations
STOCK Utrntm1Ked Dmdmdx/J
014563

  • Common name: mdx-uturn-/-
  • Genetic background: mixed; includes C57BL/10ScSn, C57BL/6, DBA/2, 129X1, and 129S1
  • Double mutant strain created by crossing strain C57BL/10ScSn-Dmdmdx/J (001801) and STOCK Utrntm1Ked/J (013158)
  • The Dmdmdx mutation is a spontaneous C-to-T transition at position 3185 (exon 23) in the dystrophin muscular dystrophy (Dmd) gene, resulting in a termination codon in place of a glutamine codon, and expression of a truncated protein (Bulfield et al. 1984, Sicinski 1989).
  • The utrophin (Utrn) null targeted mutation was created by insertion of a neomycin cassette into intron 7 (Deconinck et al. 1997)
  • Muscular dystrophy begins at 6 days of age and becomes severe by 10 weeks of age, earlier than in single Dmd mutants
  • Muscle fibers are irregularly shaped and sized, many with centralized nuclei and some lymphocyte infiltration; muscle fiber necrosis and fibrosis observed
  • Rapid and progressive loss of body weight, reduced mobility, waddling gate, muscle weakness and kyphosis by 4-6 weeks of age, premature death by 20 weeks of age

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