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Type Chemically Induced Mutation; Congenic; Mutant Strain; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Background Strain C57BL/6Ros Donor Strain C3Ha.X 25 (Pgk1a Hprta) Generation N13F13p
Generation DefinitionsDonating Investigator Dr. Verne M. Chapman (deceased), Roswell Park Memorial Institute Appearance
black
Related Genotype: a/aDescription
Mice carrying the Dmdmdx-2Cv mutation display a phenotype similar to the original Dmdmdx mutation. 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).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.
| Control | ||
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| 000664 C57BL/6J | (approximate) | |
| Considerations for Choosing Controls | ||
Strains carrying other alleles of Dmd
017929 B10.Cg-Cmahtm1Avrk Dmdmdx/PtmJ 018018 B10ScSn.Cg-Prkdcscid Dmdmdx/J 002377 B6Ros.Cg-Dmdmdx-3Cv/J 002378 B6Ros.Cg-Dmdmdx-4Cv/J 002379 B6Ros.Cg-Dmdmdx-5Cv/J 001801 C57BL/10ScSn-Dmdmdx/J 013141 D2.B10-Dmdmdx/J 018915 STOCK Terctm1Rdp Dmdmdx/J 016622 STOCK Utrntm1Jrs Dmdmdx/J 014563 STOCK Utrntm1Ked Dmdmdx/J View Strains carrying other alleles of Dmd (10 strains)
View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Cardiomyopathy, Dilated, 3b; CMD3B (DMD)
Muscular Dystrophy, Becker Type; BMD (DMD)
Muscular Dystrophy, Duchenne Type; DMD (DMD)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms provided by MGI
assigned by genotype
Dmdmdx-2Cv/Dmdmdx-2Cv
B6Ros.Cg-Dmdmdx-2Cv
- muscle phenotype
- abnormal diaphragm morphology
- mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age (MGI Ref ID J:23502)
- skeletal muscle fibrosis
- mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice (MGI Ref ID J:23502)
Dmdmdx-2Cv/Y
B6Ros.Cg-Dmdmdx-2Cv
- muscle phenotype
- abnormal diaphragm morphology
- mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age (MGI Ref ID J:23502)
- skeletal muscle fibrosis
- mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice (MGI Ref ID J:23502)
Dmdmdx-2Cv/Y
B6Ros.Cg-Dmdmdx-2Cv/J
- vision/eye phenotype
- abnormal eye electrophysiology
- derived positive (P2) responses are delayed compared to in wild-type mice (MGI Ref ID J:53822)
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
- mice exhibit skeletal muscle fiber degeneration, regeneration and sometimes fibrosis (MGI Ref ID J:9638)
- skeletal muscle fiber degeneration
- mice exhibit skeletal muscle fiber degeneration, regeneration and sometimes fibrosis (MGI Ref ID J:9638)
- impaired muscle relaxation
- electromyograms reveal peudomyotonia unlike in wild-type mice (MGI Ref ID J:9638)
- cardiovascular system phenotype
- cardiac fibrosis
- unlike wild-type, minimal to focal cardiac fibrosis is observed (MGI Ref ID J:9638)
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:
Dmdmdx-2Cv relatedMouse/Human Gene Homologs
muscular dystrophy (Duchenne and Becker)
Neurobiology Research
Muscular Dystrophy
Becker type
Duchenne type
Mouse/Human Gene Homologs
muscular dystrophy (Duchenne and Becker)
Neurobiology Research
Muscular Dystrophy
Becker type
Duchenne type
| Allele Symbol | Dmdmdx-2Cv | ||
|---|---|---|---|
| Allele Name | X linked muscular dystrophy 2, Verne Chapman | ||
| Allele Type | Chemically induced (ENU) | ||
| Common Name(s) | mdx2cv; | ||
| Mutation Made By | Dr. Verne Chapman (deceased), Roswell Park Memorial Institute | ||
| Strain of Origin | C3Ha.Cg-Hprt Pgk1 | ||
| Gene Symbol and Name | Dmd, dystrophin, muscular dystrophy | ||
| Chromosome | X | ||
| Gene Common Name(s) | BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dp427; Dp71; Duchenne muscular dystrophy; 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] | ||
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]
Danko I; Chapman V; Wolff JA. 1992. The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr Res 32(1):128-31. [PubMed: 1635838] [MGI Ref ID J:23502]
Dmdmdx-2Cv relatedDanko I; Chapman V; Wolff JA. 1992. The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr Res 32(1):128-31. [PubMed: 1635838] [MGI Ref ID J:23502]
Im WB; Phelps SF; Copen EH; Adams EG; Slightom JL; Chamberlain JS. 1996. Differential expression of dystrophin isoforms in strains of mdx mice with different mutations. Hum Mol Genet 5(8):1149-53. [PubMed: 8842734] [MGI Ref ID J:34517]
Pillers DA; Weleber RG; Green DG; Rash SM; Dally GY; Howard PL ; Powers MR ; Hood DC ; Chapman VM ; Ray PN ; Woodward WR. 1999. Effects of dystrophin isoforms on signal transduction through neural retina: genotype-phenotype analysis of duchenne muscular dystrophy mouse mutants. Mol Genet Metab 66(2):100-10. [PubMed: 10068512] [MGI Ref ID J:53822]
Animal Health Reports
Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.Colony Maintenance
Diet Information LabDiet® 5K52/5K67
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3000.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
| Pricing for International shipping destinations |
|
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
Supply Notes
- Cryorecovery - Standard.
Progeny testing is not required.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 11 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).
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Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
| Control | ||
|---|---|---|
| 000664 C57BL/6J | (approximate) | |
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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