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Genes & Alleles

Dmd;   Dmdmdx-2Cv;

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Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Mutant Strain Species laboratory mouse Background Strain C57BL/6Ros Donor Strain C3Ha.X 25 (Pgk1a Hprta) Donating Investigator Verne Chapman (deceased),   Roswell Park Memorial Institute Generation N13F13p

Appearance
black
Related Genotype: a/a

Strain Description
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 these strains are also hemizygous for Hprt^a and Pgk1^a (both are on the X chromosome).

Strain Development
This strain was created in the laboratory of Verne M. Chapman. A C57BL/6Ros female was crossed to a male of strain C3Ha.X₂₅, a double congenic strain carrying Pgk1^a (from a wild Mus musculus musculus mouse trapped in Denmark) and Hprt^a (from Mus castaneus) on a C3H/HeHa background. F1 or F2 male progeny of this cross were treated with n-ethylnitrosourea (ENU) and crossed to C57BL/10Sn-Dmd^mdx/+ females. Female offspring of these crosses that exhibited consistently elevated plasma creatine kinase levels and that carried the X-chromosome markers of their mutagenized male progenitors were bred to C57BL/10Sn-Dmd^mdx/Y males. Transmission to male progeny of the elevated plasma CK phenotype and failure of the suspected new mutations at the Dmd locus to complement the classical mdx mutation identified four new mutations of Dmd, called Dmd^mdx-2-5Cv . Each of these new mutations was subsequently backcrossed onto C57BL/6Ros.

Mammalian Phenotype Terms assigned by genotype Dmdmdx-2Cv/Dmdmdx-2Cv         B6Ros.Cg-Dmdmdx-2Cv muscle phenotype abnormal diaphragm morphology (MGI Ref ID J:23502) mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild type mice that increases with age other phenotype fibrosis (MGI Ref ID J:23502) mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild type mice that increases with age Dmdmdx-2Cv/Y         B6Ros.Cg-Dmdmdx-2Cv muscle phenotype abnormal diaphragm morphology (MGI Ref ID J:23502) mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild type mice that increases with age other phenotype fibrosis (MGI Ref ID J:23502) mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild type mice that increases with age Dmdmdx-2Cv/Y         B6Ros.Cg-Dmdmdx-2Cv/J vision/eye phenotype abnormal eye electrophysiology (MGI Ref ID J:53822) derived positive (P2) responses are delayed compared to in wild type mice The following phenotype information may relate to a genetic background differing from this JAX® Mice strain. Dmdmdx-2Cv/Y         involves: C3H/HeHa * C57BL/6Ros * C57BL/10Sn * M. m. castaneus * M. m musculus muscle phenotype abnormal skeletal muscle fiber morphology (MGI Ref ID J:9638) mice exhibit skeletal muscle fiber degeneration, regeneration and sometimes fibrosis unlike in wild type mice impaired muscle relaxation (MGI Ref ID J:9638) electromyograms reveal peudomyotonia unlike in wild type mice muscle degeneration (MGI Ref ID J:9638) mice exhibit skeletal muscle fiber degeneration, regeneration and sometimes fibrosis unlike in wild type mice cardiovascular system phenotype cardiac fibrosis (MGI Ref ID J:9638) unlike wild type, minimal to focal cardiac fibrosis is observed

Gene & Allele Details

Allele Symbol		Dmdmdx-2Cv
Allele Name		X linked muscular dystrophy 2, Verne Chapman
Common Name(s)		mdx2cv;
Mutation Made By		Verne Chapman (deceased),   Roswell Park Memorial Institute
Strain of Origin		C3Ha.Cg-Hprt1 Pgk1
Gene Symbol and Name		Dmd, dystrophin, muscular dystrophy
Chromosome		X
Gene Common Name(s)		BMD; CMD3B; DNADMD1; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dp427; Duchenne muscular dystrophy; RATDMD; X-linked muscular dystrophy; mdx; pke; pyruvate kinase expression;
Molecular Note		An A to T transversion two nucleotides 5' to the intron 42/exon 43 splice accesptor site. This mutation abolishes splicing at this site and induces aberrant splice products that do not preserve the reading frame. [MGI Ref ID J:34517]

Control Information

	Control
	000664 C57BL/6J	(approximate)
Considerations for Choosing Controls

Colony Maintenance

Diet Information	LabDiet® 5K52/5K67

Related Strains

Strains carrying other alleles of Dmd

002377	B6Ros.Cg-Dmdmdx-3Cv/J
002378	B6Ros.Cg-Dmdmdx-4Cv/J
002379	B6Ros.Cg-Dmdmdx-5Cv/J
001801	C57BL/10ScSn-Dmdmdx/J

View Strains carrying other alleles of Dmd     (4 strains)

Additional Web Information

Congenic Nomenclature

Research Applications

This mouse can be used to support research in many areas including:

Dmd^mdx-2Cv related

Mouse/Human Gene Homologs
muscular dystrophy (Duchenne and Becker)

Neurobiology Research
Neuromuscular Defects

References

Selected Reference(s)

Chapman VM; Miller DR; Armstrong D; Caskey CT. 1989. Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice. Proc Natl Acad Sci U S A 86(4):1292-6. [PubMed: 2919177]  [MGI Ref ID J:9638]

Additional References

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Price and Supply Information

Strain Name:	B6Ros.Cg-Dmdmdx-2Cv/J
Stock Number:	002388

Price Details

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Supply Details

Standard Supply	Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes	Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org. This strain is included in the Induced Mutant Resource Colony collection. Genomic DNA is available for this strain from the Mouse DNA Resource.
Licensing	See General Terms and Conditions below
Control Information	View Control Information in Strain Details.

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX^® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX^® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX^® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

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