JAX Mice Database - muscular dystrophy (Duchenne and Becker)

Search Criteria: Research Area is "Mouse/Human Gene Homologs: muscular dystrophy (Duchenne and Becker)"

JAX^® Mice Strains

Stock Number	Strain Name Strain Description	Standard Supply
001801	C57BL/10ScSn-Dmd^mdx/J	Level 2
	The X-linked dystrophin gene (Dmd) is highly expressed in muscle cells and encodes a cytoskeletal protein which localizes to the inner face of the sarcolemma. Dystrophin molecules bind to cytoskeletal F-actin and transmembrane beta-dystroglycan as part of a complex, multimolecular unit that mediates signaling between the intracellular cytoskeleton and the extracellular matrix. The structure and localization also suggest that dystrophin is important for stabilizing the plasma membrane, particularly during contraction. The mdx mutation of Dmd is recessive and heterozygous females are visually indistinguishable from wild-type mice. Females homozygous and males hemizygous for the Dmd^mdx allele retain a normal lifespan and can survive up to two years. Like human patients who suffer from one of the most common neuromuscular diseases, Duchenne muscular dystrophy (DMD), the Dmd^mdx mutants do not express dystrophin and therefore have been ..... For more information please see the full descriiption on the strain data sheet
002388	B6Ros.Cg-Dmd^mdx-2Cv/J	Repository-Cryopreserved
	Mice carrying the Dmd^mdx-2Cv mutation display a phenotype similar to the original Dmd^mdx mutation. Dmd^mdx-3Cv mutant mice display a faint dystrophin immunofluorescence in skeletal sarcolemma and skeletal muscle in contrast to the other mutants which show no dystrophin reactivity. This is similar to a group of human DMD patients. This mutant has a low frequency of revertants. The Dmd^mdx-4Cv and Dmd^mdx-5Cv strains have 10 times fewer revertants than the Dmd^mdx and Dmd^mdx-2Cv strains as viewed in quadricep cross-sections. This is not attributable to genetic background or viral infections. These reversion rate differences may be attributable to differences in the location of the point mutation. The large number of revertants in Dmd^mdx mutants has complicated the analysis of gene or cell therapies. These mutants are more useful for this purpose. All ..... For more information please see the full descriiption on the strain data sheet
002377	B6Ros.Cg-Dmd^mdx-3Cv/J	Repository-Cryopreserved
	Dmd^mdx-3Cv mutant mice display a faint dystrophin immunofluorescence in skeletal sarcolemma and skeletal muscle in contrast to the other mutants which show no dystrophin reactivity. This is similar to a group of human DMD patients. This mutant has a low frequency of revertants. The Dmd^mdx-4Cv and Dmd^mdx-5Cv strains have 10 times fewer revertants than the Dmd^mdx and Dmd^mdx-2Cv strains as viewed in quadricep cross-sections. This is not attributable to genetic background or viral infections. These reversion rate differences may be attributable to differences in the location of the point mutation. The large number of revertants in Dmd^mdx mutants has complicated the analysis of gene or cell therapies. These mutants are more useful for this purpose. All these strains are also hemizygous for Hprt^a and Pgk1^a (both are on the X chromosome).
002378	B6Ros.Cg-Dmd^mdx-4Cv/J	Repository-Cryopreserved
	The Dmd^mdx-4Cv and Dmd^mdx-5Cv strains have 10 times fewer revertants than the Dmd^mdx and Dmd^mdx-2Cv strains as viewed in quadricep cross-sections. This is not attributable to genetic background or viral infections. These reversion rate differences may be attributable to differences in the location of the point mutation. The large number of revertants in Dmd^mdx mutants has complicated the analysis of gene or cell therapies. These mutants are more useful for this purpose. All these strains are also hemizygous for Hprt^a and Pgk1^a (both are on the X chromosome).
002379	B6Ros.Cg-Dmd^mdx-5Cv/J	Repository-Cryopreserved
	Mice carrying the Dmd^mdx-5Cv mutation have 10 times fewer revertants than the Dmd^mdx and Dmd^mdx-2Cv strains as viewed in quadricep cross-sections. This is not attributable to genetic background or viral infections. These reversion rate differences may be attributable to differences in the location of the point mutation. The large number of revertants in Dmd^mdx mutants has complicated the analysis of gene or cell therapies. These mutants are more useful for this purpose. This strain is also hemizygous for Hprt^a and Pgk1^a (both are on the X chromosome).

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