Go to JAX® Mice Query Form

Strain Name:

B6Ros.Cg-Dmdmdx-3Cv/J

Stock Number:

002377

Availability:

Repository-Cryopreserved

View Pricing for USA, Canada and Mexico
View Pricing for International

General Terms and Conditions

Genes & Alleles   Dmd;   Dmdmdx-3Cv;

Product Information

Strain Details

Type JAX® GEMM® Strain - Chemically Induced Mutation
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Congenic
Type JAX® GEMM® Strain - Mutant Strain
Specieslaboratory mouse
Background Strain C57BL/6Ros
Donor Strain C3Ha.X25 (Pgk1a Hprta )
Donating Investigator Verne Chapman (deceased),   Roswell Park Memorial Institute
GenerationN?+1F14p

Appearance
black
Related Genotype: a/a

Strain Description
Dmdmdx-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 Dmdmdx-4Cv and Dmdmdx-5Cv strains have 10 times fewer revertants than the Dmdmdx and Dmdmdx-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 Dmdmdx 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 Hprta and Pgk1a (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 Pgk1a (from a wild Mus musculus musculus mouse trapped in Denmark) and Hprta (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-Dmdmdx/+ 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-Dmdmdx/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 Dmdmdx-2-5Cv . Each of these new mutations was subsequently backcrossed onto C57BL/6Ros.

Mammalian Phenotype Terms assigned by genotype

Dmdmdx-3Cv/Dmdmdx-3Cv

        B6Ros.Cg-Dmdmdx-3Cv
  • lethality-postnatal
  • postnatal lethality (MGI Ref ID J:12150)
    • fewer than expected mice survive until weaning compared to Dmdmdx mice
  • reproductive system phenotype
  • decreased litter size (MGI Ref ID J:12150)
  • reduced fertility (MGI Ref ID J:12150)
    • fewer than exected mice are produced compared to Dmdmdx mice
  • muscle phenotype
  • abnormal muscle morphology (MGI Ref ID J:12150)
    • unlike in wild type mice, myofibers contain fibers of different diameters with centrally located nuclei
    • 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
    • abnormal muscle fiber morphology (MGI Ref ID J:23502)
      • 70% of myofibers contain central nuclei unlike in wild type mice
  • 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-3Cv/Dmdmdx-3Cv

        B6Ros.Cg-Dmdmdx-3Cv/J
  • nervous system phenotype
  • abnormal Muller cell morphology (MGI Ref ID J:125566)
    • mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild type cells
  • vision/eye phenotype
  • abnormal Muller cell morphology (MGI Ref ID J:125566)
    • mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild type cells

Dmdmdx-3Cv/Y

        B6Ros.Cg-Dmdmdx-3Cv
  • lethality-postnatal
  • postnatal lethality (MGI Ref ID J:12150)
    • fewer than expected mice survive until weaning compared to Dmdmdx mice
  • reproductive system phenotype
  • decreased litter size (MGI Ref ID J:12150)
  • reduced fertility (MGI Ref ID J:12150)
    • fewer than exected mice are produced compared to Dmdmdx mice
  • muscle phenotype
  • abnormal muscle morphology (MGI Ref ID J:12150)
    • unlike in wild type mice, myofibers contain fibers of different diameters with centrally located nuclei
    • 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
    • abnormal muscle fiber morphology (MGI Ref ID J:23502)
      • 70% of myofibers contain central nuclei unlike in wild type mice
  • 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-3Cv/Y

        B6Ros.Cg-Dmdmdx-3Cv/J
  • muscle phenotype
  • abnormal muscle contractility (MGI Ref ID J:134272)
    • highest specific tetanic force is lower than in wild type mice
    • at high frequencies mice titanic force is greater than in Dmdmdx-4Cv mice
    • following eccentric contraction damage, force is reduced to unlike in wild type mice that experience only a moderate drop in force but not as severely as in Dmdmdx-4Cv mice
    • age exacerbates force losses
  • abnormal skeletal muscle morphology (MGI Ref ID J:134272)
    • skeletal muscles exhibit variation in fiber size and centrally located nuclei unlike in wild type mice
    • mice exhibit macrophage invasion into muscles unlike in wild type mice
    • increased skeletal muscle mass (MGI Ref ID J:134272)
    • increased skeletal muscle size (MGI Ref ID J:134272)
  • vision/eye phenotype
  • *normal* vision/eye phenotype (MGI Ref ID J:53822)
    • retina morphology is normal
    • abnormal Muller cell morphology (MGI Ref ID J:125566)
      • mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild type cells
    • abnormal eye electrophysiology (MGI Ref ID J:53822)
      • mice exhibit a severely attenuated b-wave amplitude compared to wild type mice
      • b-wave and OP3 wave implicit times are delayed compared to in wild type mice
      • derived positive (P2) responses are delayed compared to in wild type mice
  • behavior/neurological phenotype
  • abnormal grip strength (MGI Ref ID J:134272)
    • mice exhibit stronger grip strength than Dmdmdx-4Cv mice
  • nervous system phenotype
  • abnormal Muller cell morphology (MGI Ref ID J:125566)
    • mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild type cells

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Dmdmdx-3Cv/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 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 unlike in wild type mice
  • cardiovascular system phenotype
  • cardiac fibrosis (MGI Ref ID J:9638)
    • unlike wild type, some cardiac fibrosis is observed

Gene & Allele Details

Allele Symbol Dmdmdx-3Cv
Allele Name X linked muscular dystrophy 3, Verne Chapman
Common Name(s) mdx3cv; mdx3cv; mdx3cv; mdxcv3;
Mutation Made By Verne Chapman (deceased),   Roswell Park Memorial Institute
Strain of OriginC3Ha.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 A T to A transversion creates a novel splice acceptor site 14 bp upstream of the natural site in exon 66. Splicing at this mutant site results in the inclusion of 14 bp of intronic sequence and shifts the reading frame of the encoded mRNA. Western blotanalysis failed to detect any isoform of protein in various tissues from homozygous mutant mice. [MGI Ref ID J:12150]

Control Information

  Control
   000664 C57BL/6J (approximate)
   Controls are provided from the C57BL/6J colony (Stock No. 000664). These only provide an approximate genetic match to this C57BL/6Ros background.
 
  Considerations for Choosing Controls

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying other alleles of Dmd
002388   B6Ros.Cg-Dmdmdx-2Cv/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:

Dmdmdx-3Cv 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

Price and Supply Information

Strain Name: B6Ros.Cg-Dmdmdx-3Cv/J
Stock Number: 002377

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.

*NO Shipping Destination selected!

 

Supply Details

Standard SupplyRepository-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.

LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

The Jackson Laboratory's Genotype Promise

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.
Ordering and Purchasing Information

      Purchasing Information
      JAX® Mice Orders
      Surgical Services

Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Go to JAX® Mice Query Form

(2.15)