Strain Detail Sheet

Strain Name    :

B6.129-Sgcdtm1Kcam/Mmmh

Stock Number :

000406-MU

Gene Information

Gene Details [Including genotyping protocols]

(provided by MGI)
Allele Symbol: Sgcdtm1Kcam
Name: targeted mutation 1, Kevin P Campbell
Alteration at locus: Knockout
Gene Symbol: Sgcd
Name: sarcoglycan, delta (dystrophin-associated glycoprotein)
Chromosome: 11
Alteration at locus: Knockout

Genetic Alterations:
A targeting vector containing a neo cassette was inserted to replace exon 2, which encodes for 63 amino acids of the intracelular domain and the entire transmembrane region.

Genotype Determination:

ES Cell Line: R1 derived from 129 x C57BL/6 hybrid

Strain Description [Including phenotype, strain background, strain development and suggested control mice]

Phenotype

Homozygous Phenotype: Sgcd null mice show a phenotype consistent with human limb girdle muscular dystrophy. Pathology includes large regions of necrosis in nearly all skeletal muscles. Severe necrotic lesions are present in diaphragm of mice under 1 month of age. Mice older than 6 months of age display cardiomyopathy. Mice display irregularities of the coronary vasculature.

Heterozygous Phenotype: Heterozygotes do not show any known 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.
Sgcdtm1Kcam/Sgcdtm1Kcam
        involves: 129S1/Sv * 129X1/SvJ
  • mortality/aging
    • increased sensitivity to induced morbidity/mortality (MGI Ref ID J:57107)
      • during a treadmill stress test at 2-3 months of age, about 1/3 of homozygotes died suddenly during the exercise
    • premature death (MGI Ref ID J:57107)
      • increase in the number of spontaneous deaths at around 6 months of age
  • cardiovascular system phenotype
    • abnormal cardiovascular system physiology (MGI Ref ID J:57107)
      • cardiomyopathy (MGI Ref ID J:57107)
        • develop severe cardiomyopathy after the age of 3 months, with focal areas of myocardial ischemic-like lesions followed by fibrotic calcification and scarring of tissue
        • females that have been pregnant at least once display more widespread and advanced cardiac alterations than virgin females
        • dilated cardiomyopathy (MGI Ref ID J:57107)
      • decreased QRS amplitude (MGI Ref ID J:57107)
        • ventricular excitation (QRS amplitude and duration) is perturbed, with significantly smaller QRS amplitudes, however resting heart rate and PR intervals are normal
      • dystrophic cardiac calcinosis (MGI Ref ID J:57107)
        • exhibit granular calcium deposits in myocytes at 3 months of age
    • abnormal heart morphology (MGI Ref ID J:57107)
      • abnormal heart ventricle morphology (MGI Ref ID J:57107)
        • severe dilation of the heart ventricle
      • cardiac fibrosis (MGI Ref ID J:57107)
        • fibrosis is seen in older mice (5-6 months)
      • coronary artery stenosis (MGI Ref ID J:57107)
        • coronary vessels show multiple constrictions with pre- and poststenotic dilations as well as narrow vessels with a serrated rather than smooth contour
      • dilated cardiomyopathy (MGI Ref ID J:57107)
      • myocardial necrosis (MGI Ref ID J:57107)
        • progressive development of myocardial necrosis
        • treadmill exercise at 2-3 months of age (when no signs of cardiac muscle necrosis are seen) initiates the development of cardiac muscle necrosis
        • administration of a vascular smooth muscle relaxant prevents onset of myocardial necrosis
  • muscle phenotype
    • cardiomyopathy (MGI Ref ID J:57107)
      • develop severe cardiomyopathy after the age of 3 months, with focal areas of myocardial ischemic-like lesions followed by fibrotic calcification and scarring of tissue
      • females that have been pregnant at least once display more widespread and advanced cardiac alterations than virgin females
      • dilated cardiomyopathy (MGI Ref ID J:57107)
    • dystrophic muscle (MGI Ref ID J:57107)
      • exhibit severe muscular dystrophy
      • dystrophic changes include severe necrotic/regenerative lesions, endomysial fibrosis, fiber splitting, hypertrophy, dystrophic calcification, fatty infiltration, and increased levels of creatine kinase
    • skeletal muscle necrosis (MGI Ref ID J:57107)
      • exhibit various stages of necrosis or regeneration
      • large regions of necrosis/regeneration in the calf and thigh muscles are seen at all ages, while severe necrot ic lesions in the diaphragm are seen at 1 month of age

Strain of Origin: 129 x C57BL/6 hybrid

Strain genetic background: C57BL/6J

Research Applications

  • Cardiovascular
  • Developmental Biology
  • Models for Human Disease
  • Musculoskeletal

Strain Origin

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

Primary Reference:

  • Coral-Vazquez R, Cohn RD, Moore SA, Hill JA, Weiss RM, Davisson RL, Straub V, Barresi R, Bansal D, Hrstka RF, Williamson R, Campbell KP. Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy. Cell. 1999 Aug 20;98(4):465-74. (Medline PMID: 10481911)
  • Cohn RD, Durbeej M, Moore SA, Coral-Vazquez R, Prouty S, Campbell KP. Prevention of cardiomyopathy in mouse models lacking the smooth muscle sarcoglycan-sarcospan complex. J Clin Invest. 2001 Jan;107(2):R1-7. (Medline PMID: 11160141)

Colony and Husbandry Information

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 recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@missouri.edu.

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 [Including applicable technology transfer agreements]

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.

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
000406-MU-RESUSLitter recovered from cryo-archive
$2,022.00
Non-Profit
Litter Recovered litter1; additional fees for any special requests.

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 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 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.

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 in the fees section 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.



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.



The MMRRC is a collaborative effort, funded by grants from DPCPSI of the NIH.

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