Strain Name:

B6.Cg-Sgcatm1Kcam Tg(Ckm-SGCE)1Kcam/J

Stock Number:

009651

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Availability:

Cryopreserved - Ready for recovery

Use Restrictions Apply, see Terms of Use
This strain carries both a mouse alpha-sarcoglycan (Sgca) targeted mutation and a human epsilon-sarcoglycan (SGCE) transgene. Mice display no overt phenotype with normal behavior and normal muscle force generation. After mild exercise, the compound mutants show an exaggerated fatigue response. This strain may be useful in studies of muscular dystrophy and exercise-induced fatigue.

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 Congenic; Targeted Mutation; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
 
Donating Investigator Kevin Campbell,   University of Iowa

Description
Homozygous alpha-sarcoglycan targeted mutant mice develop a progressive pathology characteristic of limb-girdle muscular dystrophy type 2D starting at one week of age. Necrosis, regeneration, central nucleation, atrophy, hypertrophy, fiber splitting and endomysial fibrosis are observed in their muscle.

When combined with a human epsilon-sarcoglycan transgene driven by a mouse muscle creatine kinase promoter specific to skeletal and heart muscle (as in this compound mutant strain) mice display no overt phenotype with normal behavior and normal muscle force generation. After mild exercise, the compound mutants show an exaggerated fatigue response. Biochemically, the mice show normal levels of nNOS (NOS1) in muscle homogenates, but variable levels in membrane fractions. By immunofluorescence, nNOS (NOS1) is not localized to all muscle membrane fibers.

Development
The Sgca targeted allele was made by replacing exons 2 and 3 of the gene with a neomycin resistance cassette. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1- Kitl+-derived R1 embryonic stem (ES) cells. The line was backcrossed five times to C57BL/6 by the donating laboratory.

The mouse muscle creatine kinase promoter specific for skeletal and cardiac muscle drives expression of the full-length human epsilon-sarcoglycan cDNA in the transgenic portion of this strain. This is Line 1 of the transgenic line. The transgenic vector was introduced to B6SJLF1 embryos. The mutation was backcrossed twice to C57BL/6 then crossed to the Sgca line mentioned above for 13 generations by the donating laboratory.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

View Strains carrying other alleles of Ckm     (11 strains)

Strains carrying other alleles of Sgca
008275   B6.129S6-Sgcatm2Kcam/J
View Strains carrying other alleles of Sgca     (1 strain)

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.
Muscular Dystrophy, Limb-Girdle, Type 2D; LGMD2D   (SGCA)
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Myoclonic Dystonia   (SGCE)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Sgcatm1Kcam/Sgcatm1Kcam Tg(Ckm-SGCE)1Kcam/0

        involves: 129P2/OlaHsd * C57BL/6 * C57BL/6J * SJL
  • cardiovascular system phenotype
  • cardiomyopathy   (MGI Ref ID J:177563)
  • muscle phenotype
  • cardiomyopathy   (MGI Ref ID J:177563)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Neurobiology Research
Muscular Dystrophy
      Limb-Girdle type

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Sgcatm1Kcam
Allele Name targeted mutation 1, Kevin P Campbell
Allele Type Targeted (Null/Knockout)
Common Name(s) KO/KO; Sgcanull; Sgcatm594Kcam; alphaSG-;
Strain of Origin(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
ES Cell Line NameR1
ES Cell Line Strain(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Gene Symbol and Name Sgca, sarcoglycan, alpha (dystrophin-associated glycoprotein)
Chromosome 11
Gene Common Name(s) 50-DAG; 50DAG; A2; ADL; Asg; DAG2; DMDA2; LGMD2D; SCARMD1; adhalin;
Molecular Note Replacement of 902 bp including exons 2 and 3 with a neomycin cassette. No mRNA was detectable in Northern blots of total RNA derived from skeletal muscle tissue of homozygous mutants, and no protein was detected in Western blots in membrane enriched preparations of skeletal muscle. [MGI Ref ID J:49992]
 
Allele Symbol Tg(Ckm-SGCE)1Kcam
Allele Name transgene insertion 1, Kevin Campbell
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) MCKeSG;
Strain of Origin(C57BL/6 x SJL)F1
Expressed Gene SGCE, sarcoglycan, epsilon, human
Promoter Ckm, creatine kinase, muscle, mouse, laboratory
Molecular Note The mouse muscle creatine kinase promoter specific for skeletal and cardiac muscle drives expression of the full-length human epsilon sarcoglycan in this transgene construct. [MGI Ref ID J:144084]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Sgcatm1Kcam, Standard PCR
Tg(Ckm-SGCE)1Kcam, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

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]

Additional References

Sgcatm1Kcam related

Allamand V; Donahue KM; Straub V; Davisson RL; Davidson BL; Campbell KP. 2000. Early adenovirus-mediated gene transfer effectively prevents muscular dystrophy in alpha-sarcoglycan-deficient mice. Gene Ther 7(16):1385-91. [PubMed: 10981665]  [MGI Ref ID J:196642]

Bartoli M; Bourg N; Stockholm D; Raynaud F; Delevacque A; Han Y; Borel P; Seddik K; Armande N; Richard I. 2006. A mouse model for monitoring calpain activity under physiological and pathological conditions. J Biol Chem 281(51):39672-80. [PubMed: 17056592]  [MGI Ref ID J:117658]

Bartoli M; Gicquel E; Barrault L; Soheili T; Malissen M; Malissen B; Vincent-Lacaze N; Perez N; Udd B; Danos O; Richard I. 2008. Mannosidase I inhibition rescues the human alpha-sarcoglycan R77C recurrent mutation. Hum Mol Genet 17(9):1214-21. [PubMed: 18252745]  [MGI Ref ID J:133929]

Beedle AM; Nienaber PM; Campbell KP. 2007. Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex. J Biol Chem 282(23):16713-7. [PubMed: 17452335]  [MGI Ref ID J:122734]

Cassano M; Biressi S; Finan A; Benedetti L; Omes C; Boratto R; Martin F; Allegretti M; Broccoli V; Cusella De Angelis G; Comoglio PM; Basilico C; Torrente Y; Michieli P; Cossu G; Sampaolesi M. 2008. Magic-factor 1, a partial agonist of Met, induces muscle hypertrophy by protecting myogenic progenitors from apoptosis. PLoS ONE 3(9):e3223. [PubMed: 18795097]  [MGI Ref ID J:144095]

Crosbie RH; Dovico SA; Flanagan JD; Chamberlain JS; Ownby CL; Campbell KP. 2002. Characterization of aquaporin-4 in muscle and muscular dystrophy. FASEB J 16(9):943-9. [PubMed: 12087055]  [MGI Ref ID J:120469]

Crosbie RH; Lebakken CS; Holt KH; Venzke DP; Straub V; Lee JC; Grady RM; Chamberlain JS; Sanes JR; Campbell KP. 1999. Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex. J Cell Biol 145(1):153-65. [PubMed: 10189375]  [MGI Ref ID J:77615]

Dellavalle A; Maroli G; Covarello D; Azzoni E; Innocenzi A; Perani L; Antonini S; Sambasivan R; Brunelli S; Tajbakhsh S; Cossu G. 2011. Pericytes resident in postnatal skeletal muscle differentiate into muscle fibres and generate satellite cells. Nat Commun 2:499. [PubMed: 21988915]  [MGI Ref ID J:188175]

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. 1998. Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice. J Cell Biol 142(6):1461-71. [PubMed: 9744877]  [MGI Ref ID J:49992]

Fernandez K; Serinagaoglu Y; Hammond S; Martin LT; Martin PT. 2010. Mice lacking dystrophin or alpha sarcoglycan spontaneously develop embryonal rhabdomyosarcoma with cancer-associated p53 mutations and alternatively spliced or mutant Mdm2 transcripts. Am J Pathol 176(1):416-34. [PubMed: 20019182]  [MGI Ref ID J:156447]

Fiaschi T; Tedesco FS; Giannoni E; Diaz-Manera J; Parri M; Cossu G; Chiarugi P. 2010. Globular adiponectin as a complete mesoangioblast regulator: role in proliferation, survival, motility, and skeletal muscle differentiation. Mol Biol Cell 21(6):848-59. [PubMed: 20089845]  [MGI Ref ID J:165080]

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]

Kobuke K; Piccolo F; Garringer KW; Moore SA; Sweezer E; Yang B; Campbell KP. 2008. A Common Disease-Associated Missense Mutation in Alpha-Sarcoglycan Fails to Cause Muscular Dystrophy in Mice. Hum Mol Genet :. [PubMed: 18252746]  [MGI Ref ID J:130252]

Lancioni A; Luisa Rotundo I; Monique Kobayashi Y; D'Orsi L; Aurino S; Nigro G; Piluso G; Acampora D; Cacciottolo M; Campbell KP; Nigro V. 2011. Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex. Hum Mol Genet 20(23):4644-54. [PubMed: 21890494]  [MGI Ref ID J:177563]

Laure L; Suel L; Roudaut C; Bourg N; Ouali A; Bartoli M; Richard I; Daniele N. 2009. Cardiac ankyrin repeat protein is a marker of skeletal muscle pathological remodelling. FEBS J 276(3):669-84. [PubMed: 19143834]  [MGI Ref ID J:147891]

Quattrocelli M; Palazzolo G; Floris G; Schoffski P; Anastasia L; Orlacchio A; Vandendriessche T; Chuah MK; Cossu G; Verfaillie C; Sampaolesi M. 2011. Intrinsic cell memory reinforces myogenic commitment of pericyte-derived iPSCs. J Pathol 223(5):593-603. [PubMed: 21341275]  [MGI Ref ID J:170841]

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. 2003. Cell therapy of alpha-sarcoglycan null dystrophic mice through intra-arterial delivery of mesoangioblasts. Science 301(5632):487-92. [PubMed: 12855815]  [MGI Ref ID J:196640]

Turk R; Sterrenburg E; van der Wees CG; de Meijer EJ; de Menezes RX; Groh S; Campbell KP; Noguchi S; van Ommen GJ; den Dunnen JT; 't Hoen PA. 2006. Common pathological mechanisms in mouse models for muscular dystrophies. FASEB J 20(1):127-9. [PubMed: 16306063]  [MGI Ref ID J:104560]

Vignier N; Amor F; Fogel P; Duvallet A; Poupiot J; Charrier S; Arock M; Montus M; Nelson I; Richard I; Carrier L; Servais L; Voit T; Bonne G; Israeli D. 2013. Distinctive serum miRNA profile in mouse models of striated muscular pathologies. PLoS One 8(2):e55281. [PubMed: 23418438]  [MGI Ref ID J:199417]

Xu R; DeVries S; Camboni M; Martin PT. 2009. Overexpression of Galgt2 reduces dystrophic pathology in the skeletal muscles of alpha sarcoglycan-deficient mice. Am J Pathol 175(1):235-47. [PubMed: 19498002]  [MGI Ref ID J:150058]

Tg(Ckm-SGCE)1Kcam related

Lancioni A; Luisa Rotundo I; Monique Kobayashi Y; D'Orsi L; Aurino S; Nigro G; Piluso G; Acampora D; Cacciottolo M; Campbell KP; Nigro V. 2011. Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex. Hum Mol Genet 20(23):4644-54. [PubMed: 21890494]  [MGI Ref ID J:177563]

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 & HusbandryThe donating investigator crosses homozygous Sgca targeted mutant/hemizygous SGCE transgenic males with homozygous Sgca targeted mutant females to maintain a colony.

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 will fulfill 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 will fulfill 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
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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