Strain Name:
B10ScSn.FVB-Dmdmdx Tg(ACTA1-DMD*)34Q6Dua/Mmmh
Stock Number:
037527-MU
Citation ID:
RRID:MMRRC_037527-MU
Other Names:
YL101 (Line 34 Q6), YL101

Strain Information

Dmdmdx
Name: dystrophin, muscular dystrophy; X linked muscular dystrophy
Synonyms: pke, mdx, pyruvate kinase expression
Type: Allele
Species: Mus musculus (mouse)
Chromosome: X
Alteration at locus: Spontaneous Mutation
Dmd
Name: dystrophin, muscular dystrophy
Synonyms: Duchenne muscular dystrophy, mdx, pke, X-linked muscular dystrophy, Dp427, Dp71, dys
Type: Gene
Species: Mus musculus (mouse)
Chromosome: X
Alteration at locus: Spontaneous Mutation
NCBI: 13405
HGNC: HGNC:2928
Homologene: 20856
DMD
Name: dystrophin
Type: Gene
Species: Homo sapiens (human)
Chromosome: X
Alteration at locus: Spontaneous Mutation
ACTA1
Name: actin, alpha 1, skeletal muscle
Type: Gene
Species: Homo sapiens (human)
Chromosome:
Alteration at locus: Spontaneous Mutation
Tg(ACTA1-DMD*)34Q6Dua
Name: transgene insertion 34Q6, Dongsheng Duan
Type: Transgene
Species: Homo sapiens (human)
Chromosome: unknown
Alteration at locus: Spontaneous Mutation
Genetic Alterations
The transgene was developed from the full-length human dystrophin gene (with encoding regions of hinge 2 through spectrin-like repeat 15, repeat 18 through repeat 23, and C-terminal domains deleted). It differs from other minimized dystrophins in that it still contains the coding regions for the spectrin-like repeats 16 and 17 responsible for nNOS recruitment to the sarcolemma. Endogenous Dmd carries a premature stop codon mutation.

HGVS nomenclature:
  • Genbank RefSeq - mRNA: NM_007868.6
  • Genbank RefSeq, protein: NP_031894.1
  • Variant, nucleic acid level: c.2983C>T
  • Variant, amino acid level, predicted: p.Gln955*
    Check this variant: LUMC Mutalyzer
Genotype Determination
Phenotype

Homozygous: The homozygous model of this strain exhibits restored skeletal muscle function and blood perfusion to skeletal muscles. It also shows improved histopathology of the muscle compared to the mdx mouse.

Heterozygous: Not tested

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
  • Dystrophin/deficiency
  • Male
  • Mice
  • Mice, Inbred mdx
  • Mice, Knockout
  • Muscle Contraction/physiology
  • Muscle, Skeletal/physiopathology
  • Muscular Dystrophies, Limb-Girdle/metabolism
  • Muscular Dystrophies, Limb-Girdle/physiopathology
  • Muscular Dystrophy, Animal/metabolism
  • Muscular Dystrophy, Animal/physiopathology
  • Muscular Dystrophy, Duchenne/metabolism
  • Muscular Dystrophy, Duchenne/physiopathology
  • Nitric Oxide Synthase Type I/deficiency
  • Nitric Oxide Synthase Type I/physiology
  • Oxidative Stress/physiology
  • Sarcoglycans/deficiency
  • Sarcolemma/enzymology
  • Crosses, Genetic
  • Female
  • Genetic Linkage
  • 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
The strain was developed initially by random transgenic insertion on the FVB/NJ background, then backcrossed to the C57BL/10 background with the mdx mutation for more than nine generations.
Suggested Control Mice
Wild-type littermates or C57BL/10 mice
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.
  • Cardiovascular
  • Cell Biology
  • Immunology and Inflammation
  • Models for Human Disease
Donor

Primary Reference

Li D, Yue Y, Lai Y, Hakim CH, Duan D. Nitrosative stress elicited by nNOS delocalization inhibits muscle force in dystrophin-null mice. J Pathol. 2011 Jan;223(1):88-98. (Medline PMID: 021125668)

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

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.
Coat Color
Black
Eye
Black
MMRRC Breeding System
Backcross
Generation
N9+ (C57BL/10ScSn-Dmdmdx/J, JAX Strain #001801)
Overall Breeding Performance
Excellent
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: Yes Yes
Age Reproductive Decline: Undetermined Undetermined
Bred to Homozygosity
Yes
Average litter size
5-9
Recommended wean age
3 weeks
Average Pups Weaned
5-9

Order Request Information

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.

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.

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

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
037527-MU-SPERM Cryo-preserved spermatozoa $437.00 / Non-Profit Aliquot Approximate quantity3
037527-MU-RESUS Litter recovered from cryo-archive $2,624.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

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.