Strain Detail Sheet

Strain Name:
B10.Cg-Tg(DMD)72Thoen Dmdmdx/DuaMmmh
Stock Number:
050929-MU
Citation ID:
RRID:MMRRC_050929-MU
Other Names:
hDMD/Mdx

Strain Information

Gene Details

Dmdmdx
Name: dystrophin, muscular dystrophy; X linked muscular dystrophy
Synonyms: pyruvate kinase expression, mdx, pke
Type: Allele
Species: Mus musculus (mouse)
Chromosome: X
Alteration at locus: Spontaneous Mutation
Dmd
Name: dystrophin, muscular dystrophy
Synonyms: Dp71, X-linked muscular dystrophy, Duchenne muscular dystrophy, Dp427, dys, pke, mdx
Type: Gene
Species: Mus musculus (mouse)
Chromosome: X
Alteration at locus: Spontaneous Mutation
NCBI: 13405
HGNC: HGNC:2928
Homologene: 20856
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Tg(DMD)72Thoen
Name: transgene insertion 72, Peter 't Hoen
Synonyms: hDMD
Type: Allele
Species: Multi-species
Chromosome: 5
Alteration at locus: Transgenic
DMD
Name: dystrophin
Type: Gene
Species: Homo sapiens (human)
Chromosome: X
Alteration at locus: Transgenic
Genetic Alterations
The human DMD (hDmd) transgene is a 2.7-Mbp yeast artificial chromosome (YAC) sequence containing the full-length 2.3 Mbp human dystrophin gene (DMD). This transgene has a single copy integration into mouse chromosome 5.
Genotype Determination
ES Cell Line
Not specified

Strain Description

Phenotype
The animals contain a YAC expressing the full-length, human dystrophin gene (DMD). Additionally, they have been crossed with Mdx mice containing a spontaneous stop mutation in exon 23 of the mouse dystrophin gene (Dmd).
Mammalian Phenotype Terms
Allelic Composition: Dmdmdx/Dmdmdx (Genetic Background: C57BL/10ScSn )
Allelic Composition: Dmdmdx/Dmdmdx (Genetic Background: C57BL/10ScSn-Dmdmdx/J )
Allelic Composition: Dmdmdx/Dmdmdx (Genetic Background: C57BL/10ScSnJ )
Allelic Composition: Dmdmdx/Dmdmdx (Genetic Background: involves: C57BL/10ScSn )
Allelic Composition: Dmdmdx/Dmdmdx (Genetic Background: involves: C57BL/10ScSn * CB17 )
Allelic Composition: Dmdmdx/Dmd+ (Genetic Background: involves: 129S4/SvJae * C57BL/10ScSn )
Allelic Composition: Dmdmdx/Dmd+ (Genetic Background: involves: C57BL/10ScSn )
MeSH Terms
  • Animals
  • Blastocyst/cytology
  • Blastocyst/metabolism
  • Chromosomes/genetics
  • Chromosomes/metabolism
  • Crosses, Genetic
  • Drug Evaluation, Preclinical
  • Dystrophin/biosynthesis
  • Dystrophin/genetics
  • Gene Expression Regulation/genetics
  • Gene Transfer Techniques
  • Genomic Instability/genetics
  • Humans
  • Mice
  • Mice, Inbred mdx/genetics
  • Mice, Inbred mdx/metabolism
  • Mice, Transgenic/genetics
  • Mice, Transgenic/metabolism
  • Muscle, Skeletal/cytology
  • Muscle, Skeletal/metabolism
  • Muscular Dystrophy, Duchenne/drug therapy
  • Muscular Dystrophy, Duchenne/genetics
  • Muscular Dystrophy, Duchenne/metabolism
  • Organ Specificity/genetics
  • Utrophin/genetics
  • Utrophin/metabolism
  • Female
  • Genetic Linkage
  • Male
  • Mice, Inbred C57BL
  • Mice, Mutant Strains
  • Muscles/enzymology
  • Muscular Dystrophy, Animal/enzymology
  • Muscular Dystrophy, Animal/genetics
  • Muscular Dystrophy, Animal/pathology
  • Mutation
  • Pyruvate Kinase/genetics
  • Pyruvate Kinase/metabolism
  • X Chromosome
Strain Development
This strain was developed by crossing the animals harboring the Tg(DMD)72Thoen allele ("hDMD" - developed on a D2129P2F1 background and backcrossed three times to C57BL/6J) with C57BL/10ScSn-Dmdmdx mice. At each generation, mice positive for the hDMD mutation were crossed again with Dmdmdx for more than 6 generations.
Suggested Control Mice
C57BL/10ScSn-Tg(DMD)72Thoen (MMRRC:50930)

MMRRC Genetic QC

MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@missouri.edu. Older strains may not have this information.

Research Applications

  • Models for Human Disease

Strain Origin

Donor
Johan T. den Dunnen, Ph.D., Leiden University Medical Center.
Dongsheng Duan, Ph.D., University of Missouri.
Karen J. Moore, Ph.D.
Primary Reference

't Hoen PA, de Meijer EJ, Boer JM, Vossen RH, Turk R, Maatman RG, Davies KE,van Ommen GJ, van Deutekom JC, den Dunnen JT. Generation and characterization of transgenic mice with the full-length human DMD gene. J Biol Chem. 2008 Feb29;283(9):5899-907. Epub 2007 Dec 13. (Medline PMID: 18083704)

Bulfield G, Siller WG, Wight PA, Moore KJ. X chromosome-linked musculardystrophy (mdx) in the mouse. Proc Natl Acad Sci U S A. 1984 Feb;81(4):1189-92.(Medline PMID: 6583703)

Colony and Husbandry Information

Health Status Report

Colony Surveillance Program and Current Health Reports

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@missouri.edu.

Appearance

Coat Color
Black
Eye
Black

Breeding

MMRRC Breeding System
Backcross
Generation
N6+ (C57BL/10ScSn-Dmdmdx)
Overall Breeding Performance
Excellent

Reproductive Statistics

NOTE: "Hemizygote" as used here refers to males carrying a mutation on the X Chromosome or mice of either sex carrying an inserted transgene with no homologous allele on the other chromosome.
Viability and Fertility: Female Male Comments
Homozygotes are viable: Yes Yes
Homozygotes are fertile: Yes Yes
Hetero/Hemizygotes are fertile: N/A N/A
Age Reproductive Decline: Undetermined Undetermined
Average litter size
4-6
Recommended wean age
3 Weeks
Average Pups Weaned
4-6

Order Request Information

Availability Level

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

Conditions of Distribution

The donor or their institution limits the distribution to non-profit institutions only.

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

Fees

- Products for this strain are Not Yet Available for Ordering
- If you register interest in this strain, you will be notified when it becomes available for ordering.
To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.