Strain Detail Sheet

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.

Sgca^tm1Kcam/Sgca^tm1Kcam

        involves: 129S1/Sv * 129X1/SvJ

• muscle phenotype
  • abnormal muscle contractility (MGI Ref ID J:49992)
    • the specific P_o of the EDL muscle is 31% lower than that of controls, while in soleus muscle, the P_o 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:

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)

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

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.

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MMRRC Item # Description	Distribution Fee/unit (US $)	Units	Notes
000425-MU-RESUS Litter recovered from cryo-archive	$2,022.00 Non-Profit	Litter	Recovered litter1; additional fees for any special requests.
000425-MU-SPERM Cryo-preserved spermatozoa	$437.00 Non-Profit	Aliquot	Approximate quantity.2
000425-MU-EMBRYO Cryo-preserved embryos	$1,038.00 Non-Profit	Aliquot	Approximate quantity3: 20-40 embryos / aliquot

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.