Strain Name:

B6;129-Dtnatm1Jrs/J

Stock Number:

010976

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Cryopreserved - Ready for recovery

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Muscles of Dtna (Dtna, dystrobrevin alpha) homozygous mice appear histologically normal during the first two postnatal weeks, but become dystrophic by 1 month of age. Mutant mice exhibit milder pathology than humans with Duchenne dystrophy and never develop the early myofibrosis characteristic of the human disease.

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Mutant Stock; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationN1F?pN1
Generation Definitions
 
Donating Investigator Kevin Campbell,   University of Iowa

Description
None of the known alternatively spliced forms of Dtna (Dtna, dystrobrevin alpha) is detectable in non-synaptic portions of skeletal muscles or brains of homozygous mutants, and no compensation by beta-dystrobrevin is detected. Muscles of homozygous mice appear histologically normal during the first two postnatal weeks, but become dystrophic by 1 month of age. Pathological findings include small groups of degenerating myofibers and infiltrating monocytes. Regenerating fibers, characterized by expression of the embryonic myosin heavy chain and central nuclei, are also present. Degenerating muscle cells are usually grouped in clusters of 3-10 fibers. Histologically, the myopathy of homozygous mice resemble that of Dmdmdx (dystrophin, muscular dystrophy; X linked muscular dystrophy) mice, although Dtna homozygous mutants have fewer centrally nucleated fibres. In these mice, as in mdx mice, the diaphragm is the most severely affected skeletal muscle. Overall, however, both Dtna and mdx mutant mice exhibit milder pathology than humans with Duchenne dystrophy and never develop the early myofibrosis characteristic of the human disease.

Development
Exon 3 of the targeted gene was deleted and replaced with a neomycin resistance cassette. A 129/SvJ-derived embryonic stem cell line was used to make the mutation. The strain was backcrossed once to C57BL/6 and was maintained on a mixed 129 and C57BL/6 background by the donating laboratory.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

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.
Left Ventricular Noncompaction 1; LVNC1   (DTNA)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Dtnatm1Jrs/Dtnatm1Jrs

        involves: 129X1/SvJ * C57BL/6
  • muscle phenotype
  • cardiomyopathy
    • 61% exhibit mild cardiomyopathy at 1 month, including mononuclear-cell infiltration and fibrosis, associated with plaques that are scattered through both ventricles, however hearts are not dilated or hypertrophic   (MGI Ref ID J:59675)
  • dystrophic muscle
    • develop mild skeletal dystrophy by 1 month of age; see small groups of degenerating myofibers and infiltrating monocytes   (MGI Ref ID J:59675)
    • the diaphragm is the most severely affected skeletal muscle   (MGI Ref ID J:59675)
    • muscle fibers are less structurally compromised than in Dmdmdx mice and appear to maintain the dystrophin-containing glycoprotein complex   (MGI Ref ID J:59675)
    • about 40% of fibers are dystrophic   (MGI Ref ID J:60776)
  • myocardial fiber degeneration
    • degeneration of myocytes   (MGI Ref ID J:59675)
  • cardiovascular system phenotype
  • cardiac fibrosis   (MGI Ref ID J:59675)
  • cardiomyopathy
    • 61% exhibit mild cardiomyopathy at 1 month, including mononuclear-cell infiltration and fibrosis, associated with plaques that are scattered through both ventricles, however hearts are not dilated or hypertrophic   (MGI Ref ID J:59675)
  • myocardial fiber degeneration
    • degeneration of myocytes   (MGI Ref ID J:59675)
  • nervous system phenotype
  • abnormal neuromuscular synapse morphology
    • distribution of acetylcholine receptors within synapse branches is abnormal, with a patchy or granular distribution, however movement is normal   (MGI Ref ID J:60776)
    • derangement of the postsynaptic membrane, with a 50% reduction in the density of junctional folds and altered distribution of electron-dense material   (MGI Ref ID J:60776)
    • postsynaptic apparatus is fragmented into discrete boutons   (MGI Ref ID J:60776)
  • behavior/neurological phenotype
  • decreased grip strength
    • single knockout mutants perform more poorly than controls but better than double knockouts with DtnbGt(OST109050)Lex   (MGI Ref ID J:106640)
  • impaired coordination
    • single knockout mutants perform more poorly than controls but better than double knockouts with DtnbGt(OST109050)Lex   (MGI Ref ID J:106640)

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Dtnatm1Jrs
Allele Name targeted mutation 1, Joshua R Sanes
Allele Type Targeted (Null/Knockout)
Common Name(s) adbn -; alphaDB-; alphaDbKO;
Mutation Made By Joshua Sanes,   Harvard University
Strain of Origin129X1/SvJ
Gene Symbol and Name Dtna, dystrobrevin alpha
Chromosome 18
Gene Common Name(s) 2210407P21Rik; 87K protein; A0; D18S892E; DRP3; DTN; DTN-A; Dtn; Dtna-ps1; LVNC1; RGD1561985; RIKEN cDNA 2210407P21 gene; a-DB-1; adbn; alpha-dystrobrevin; dystrobrevin;
Molecular Note A neomycin resistance cassette replaced a 2.5 kb segment containing exon 3. The deleted sequence corresponds to a region in the protein that is found in the isoforms alpha-DB-1 and alpha DB-2, the only isoforms known to be expressed in muscle. However,immunohistochemical analyses of skeletal muscle and brain did not detect any of the known alternatively spliced isoforms in homozygous mutant mice. [MGI Ref ID J:59675]

Genotyping

Genotyping Information

Genotyping Protocols

Dtnatm1Jrs, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Grady RM; Grange RW; Lau KS; Maimone MM; Nichol MC; Stull JT; Sanes JR. 1999. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies. Nat Cell Biol 1(4):215-20. [PubMed: 10559919]  [MGI Ref ID J:59675]

Additional References

Dtnatm1Jrs related

Adams ME; Tesch Y; Percival JM; Albrecht DE; Conhaim JI; Anderson K; Froehner SC. 2008. Differential targeting of nNOS and AQP4 to dystrophin-deficient sarcolemma by membrane-directed {alpha}-dystrobrevin. J Cell Sci 121(Pt 1):48-54. [PubMed: 18057022]  [MGI Ref ID J:130745]

Albrecht DE; Sherman DL; Brophy PJ; Froehner SC. 2008. The ABCA1 cholesterol transporter associates with one of two distinct dystrophin-based scaffolds in Schwann cells. Glia 56(6):611-8. [PubMed: 18286648]  [MGI Ref ID J:156278]

Bragg AD; Amiry-Moghaddam M; Ottersen OP; Adams ME; Froehner SC. 2006. Assembly of a perivascular astrocyte protein scaffold at the mammalian blood-brain barrier is dependent on alpha-syntrophin. Glia 53(8):879-90. [PubMed: 16609960]  [MGI Ref ID J:156125]

Bunnell TM; Jaeger MA; Fitzsimons DP; Prins KW; Ervasti JM. 2008. Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. PLoS ONE 3(7):e2604. [PubMed: 18596960]  [MGI Ref ID J:138017]

Grady RM; Akaaboune M; Cohen AL; Maimone MM; Lichtman JW; Sanes JR. 2003. Tyrosine-phosphorylated and nonphosphorylated isoforms of alpha-dystrobrevin: roles in skeletal muscle and its neuromuscular and myotendinous junctions. J Cell Biol 160(5):741-52. [PubMed: 12604589]  [MGI Ref ID J:82177]

Grady RM; Wozniak DF; Ohlemiller KK; Sanes JR. 2006. Cerebellar synaptic defects and abnormal motor behavior in mice lacking alpha- and beta-dystrobrevin. J Neurosci 26(11):2841-51. [PubMed: 16540561]  [MGI Ref ID J:106640]

Grady RM; Zhou H; Cunningham JM; Henry MD; Campbell KP; Sanes JR. 2000. Maturation and maintenance of the neuromuscular synapse: genetic evidence for roles of the dystrophin--glycoprotein complex. Neuron 25(2):279-93. [PubMed: 10719885]  [MGI Ref ID J:60776]

Hanft LM; Bogan DJ; Mayer U; Kaufman SJ; Kornegay JN; Ervasti JM. 2007. Cytoplasmic gamma-actin expression in diverse animal models of muscular dystrophy. Neuromuscul Disord 17(7):569-74. [PubMed: 17475492]  [MGI Ref ID J:124551]

Kobayashi YM; Rader EP; Crawford RW; Iyengar NK; Thedens DR; Faulkner JA; Parikh SV; Weiss RM; Chamberlain JS; Moore SA; Campbell KP. 2008. Sarcolemma-localized nNOS is required to maintain activity after mild exercise. Nature 456(7221):511-5. [PubMed: 18953332]  [MGI Ref ID J:144084]

Martinez-Pena Y Valenzuela I; Mouslim C; Pires-Oliveira M; Adams ME; Froehner SC; Akaaboune M. 2011. Nicotinic Acetylcholine Receptor Stability at the NMJ Deficient in alpha-Syntrophin In Vivo. J Neurosci 31(43):15586-96. [PubMed: 22031904]  [MGI Ref ID J:177264]

Martinez-Pena y Valenzuela I; Akaaboune M. 2007. Acetylcholinesterase mobility and stability at the neuromuscular junction of living mice. Mol Biol Cell 18(8):2904-11. [PubMed: 17538015]  [MGI Ref ID J:128333]

Oh HJ; Abraham LS; van Hengel J; Stove C; Proszynski TJ; Gevaert K; DiMario JX; Sanes JR; van Roy F; Kim H. 2012. Interaction of alpha-catulin with dystrobrevin contributes to integrity of dystrophin complex in muscle. J Biol Chem 287(26):21717-28. [PubMed: 22577143]  [MGI Ref ID J:192994]

Patton BL; Cunningham JM; Thyboll J; Kortesmaa J; Westerblad H; Edstrom L; Tryggvason K; Sanes JR. 2001. Properly formed but improperly localized synaptic specializations in the absence of laminin alpha4. Nat Neurosci 4(6):597-604. [PubMed: 11369940]  [MGI Ref ID J:77657]

Pawlikowski BT; Maimone MM. 2008. alpha-Dystrobrevin isoforms differ in their colocalization with and stabilization of agrin-induced acetylcholine receptor clusters. Neuroscience 154(2):582-94. [PubMed: 18468804]  [MGI Ref ID J:139404]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryWhen maintained in a live colony, heterozygotes or homozygotes may be bred. Homozygotes are reported to be poor breeders and may not start breeding until after 20 weeks of age.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
Animals Provided

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 willfulfill 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 10 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 View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
Animals Provided

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 willfulfill 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 10 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).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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