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Strain Detail Sheet


Strain Name: B6.129-Sgcatm1Kcam/Mmmh

Stock Number: 000425-MU

Other Names:

Gene Details: (provided by MGI)

Allele Symbol: Sgcatm1Kcam
Name: sarcoglycan, alpha (dystrophin-associated glycoprotein); targeted mutation 1, Kevin P Campbell
Alteration at locus: Targeted Mutation

Genetic Alterations:
Two fragments carrying exon 1 and a portion of 5' and 3' contiguougus inton were inserted into pPNT. A 9-Kb fragment including a portion of intron 3 and exon 4-exon 9 was inserted into XhoI-NotI cut plasmid. The correct targeting replaced exon 2, exon 3, 571 bp of intron 1, and 65 bp of intron 3. This included a neomycin resistance gene in opposite transcriptional orientation.

Genotype Determination:

ES Cell Line: R1

Strain Description

Phenotype:
Homozygote: Sgca-cull mice show phenotype similar to dystrophin-deficient mdx mice. Pathology includes necrosis, fiber regeneration and central nucleation. Atrophy, hypertrophy, fiber splitting, and endomysial fibrosis is also present in Sgca-null muscle. Sgca-null mice older than 8 weeks of age show dystrophic calcification associated with myocyte necrosis. Fatty infiltration was evident in the mice.

Heterozygote: Do not show any morphological abnormalities.


Mammalian Phenotype Terms:(provided by MGI)      Extend all MPTs
      assigned by genotype

The following phenotype information may relate to one or more alleles on a genetic background differing from this MMRRC strain.
Sgcatm1Kcam/Sgcatm1Kcam
        involves: 129S1/Sv * 129X1/SvJ
  • muscle phenotype
    • abnormal muscle contractility (MGI Ref ID J:49992)
      • the specific Po of the EDL muscle is 31% lower than that of controls, while in soleus muscle, the Po is 39% greater
      • resistance to passive muscle stretch is 75% greater than in controls
    • abnormal muscle fiber morphology (MGI Ref ID J:49992)
      • earliest pathological changes in skeletal muscles are widely scattered clusters of necrotic myocytes or regenerating myocytes with internally placed nuclei; such clusters increased in number and size as mice age
      • fiber splitting is observed in some mutant muscle
      • abnormal sarcolemma morphology (MGI Ref ID J:49992)
        • membrane damage is exhibited in muscles as shown by uptake of Evans blue dye in 4-20 week-old mice, and by elevated serum levels of creatine kinase and muscle-specific pyruvate kinase activity in 7-10 week-old animals
      • abnormal skeletal muscle fiber morphology (MGI Ref ID J:133929)
        • centrally nucleated skeletal muscle fibers (MGI Ref ID J:49992)
          • at 8 days of age, between 1 and 2.5% of sural triceps and diaphragm myocytes respectively have central nuclei; by 8-16 weeks, more than 70% (as high as 99%) of myocytes have central nuclei
    • dystrophic muscle (MGI Ref ID J:49992)
      • other dystrophic changes including atrophy, hypertrophy, and endomysial fibrosis are observed in mutant muscle; fatty infiltration is present in some muscles of 16 week old animals
    • increased extensor digitorum longus weight (MGI Ref ID J:49992)
      • EDL muscle weight is 40% greater than controls
    • increased soleus weight (MGI Ref ID J:49992)
      • soleus muscle weight is 62% greater than in controls
    • muscle calcification (MGI Ref ID J:49992)
      • dystrophic calcification is noted in some muscles in association with myocyte necrosis in mice 8 weeks or older
  • homeostasis/metabolism phenotype
    • abnormal circulating pyruvate kinase level (MGI Ref ID J:49992)
      • elevated muscle-specific pyruvate kinase activity in serum of 7-10 week-old animals
    • increased circulating creatine kinase level (MGI Ref ID J:130252)
      • mutants show significantly elevated levels of creatine kinase in the blood, relative to wild-type
    • increased circulating creatine kinase level (MGI Ref ID J:49992)
      • elevated serum levels of creatine kinase activity in 7-10 week-old animals

Founder genetic background: 129xC57BL/6

Strain genetic background: C57BL/6J

Strain Development:

Research Applications

Strain Origin

Donor: Kevin P. Campbell, Ph.D., University of Iowa

Primary Reference:

Duclos F, Straub V, Moore SA, Venzke DP, Hrstka RF, Crosbie RH, Durbeej M, Lebakken CS, Ettinger AJ, van der Meulen J, Holt KH, Lim LE, Sanes JR, Davidson BL, Faulkner JA, Williamson R, Campbell KP. Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice. J Cell Biol. 1998 Sep 21;142(6):1461-71. (Medline PMID: 9744877)

Sampaolesi M, Torrente Y, Innocenzi A, Tonlorenzi R, D'Antona G, Pellegrino MA, Barresi R, Bresolin N, De Angelis MG, Campbell KP, Bottinelli R, Cossu G. Cell therapy of alpha-sarcoglycan null dystrophic mice through intra-arterial delivery of mesoangioblasts. Science. 2003 Jul 25;301(5632):487-92. Epub 2003 Jul 10. (Medline PMID: 12855815)

Allamand V, Donahue KM, Straub V, Davisson RL, Davidson BL, Campbell KP. Early adenovirus-mediated gene transfer effectively prevents muscular dystrophy in alpha-sarcoglycan-deficient mice. Gene Ther. 2000 Aug;7(16):1385-91. (Medline PMID: 10981665)

Special Considerations

Mice with decreased mobility may benefit from gruel as food/water source.

Health Status Report

Mice recovered from a cryo-archive will have health surveillance performed on the resuscitated animals.

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.

Conditions of Distribution:

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:

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 $)
Units Notes
000425-MU-RESUSLitter recovered from cryo-archive
$2,022.00Non-Profit
Litter Recovered litter1; additional fees for any special requests.
000425-MU-SPERMCryo-preserved spermatozoa
$437.00Non-Profit
Aliquot Approximate quantity.2
000425-MU-EMBRYOCryo-preserved embryos
$1,038.00Non-Profit
Aliquot Approximate quantity3: 20-40 embryos / aliquot

Control Mice:

  • Wildtype littermates

1 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 breedable 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 sufficient material (volume and concentration) for at least two IVF or several artificial inseminations (based on our procedures). The MMRRC makes no guarantee concerning success of these procedures when performed outside the MMRRC facilities.

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

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 above). 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.


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Last Modified: April 18, 2011