Strain Name:
B6.129-Sgcatm1Kcam/Mmmh
Stock Number:
000425-MU
Citation ID:
RRID:MMRRC_000425-MU

Gene Information

Sgcatm1Kcam
Name: sarcoglycan, alpha (dystrophin-associated glycoprotein); targeted mutation 1, Kevin P Campbell
Synonyms: alphaSG-, KO/KO, Sgcatm594Kcam, Sgcanull
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 11
Alteration at locus: Targeted Mutation
Sgca
Name: sarcoglycan, alpha (dystrophin-associated glycoprotein)
Synonyms: adhalin, 50DAG, Asg
Type: Gene
Species: Mus musculus (mouse)
Chromosome: 11
Alteration at locus: Targeted Mutation
NCBI: 20391
Homologene: 9
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 derived from (129X1/SvJ x 129S1/Sv)F1-Kitl+
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
Allelic Composition: Sgcatm1Kcam/Sgcatm1Kcam (Genetic Background: involves: 129S1/Sv * 129X1/SvJ )

MeSH Terms
  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Carrier Proteins/biosynthesis
  • Carrier Proteins/physiology
  • Cytoskeletal Proteins/deficiency
  • Cytoskeletal Proteins/genetics
  • DNA, Complementary
  • Disease Progression
  • Dystrophin/metabolism
  • Gene Transfer Techniques
  • Glycoproteins/metabolism
  • Membrane Glycoproteins/deficiency
  • Membrane Glycoproteins/genetics
  • Membrane Proteins/biosynthesis
  • Membrane Proteins/physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Molecular Sequence Data
  • Muscle Contraction
  • Muscular Dystrophy, Animal/etiology
  • Muscular Dystrophy, Animal/physiopathology
  • Neoplasm Proteins
  • Sarcoglycans
  • Sarcolemma/metabolism
  • Blood Vessels/cytology
  • Blood Vessels/embryology
  • Cell Differentiation
  • Cell Line
  • Cell Movement
  • Cytoskeletal Proteins/metabolism
  • Endothelium, Vascular/physiology
  • Female
  • Femoral Artery
  • Genetic Vectors
  • Lentivirus/genetics
  • Locomotion
  • Male
  • Membrane Glycoproteins/metabolism
  • Mesoderm/cytology
  • Mice, Transgenic
  • Muscle Fibers, Skeletal/cytology
  • Muscle Fibers, Skeletal/physiology
  • Muscle, Skeletal/cytology
  • Muscle, Skeletal/metabolism
  • Muscle, Skeletal/pathology
  • Muscle, Skeletal/physiology
  • Muscular Dystrophy, Animal/metabolism
  • Muscular Dystrophy, Animal/pathology
  • Muscular Dystrophy, Animal/therapy
  • Regeneration
  • Stem Cell Transplantation
  • Stem Cells/physiology
  • Transfection
  • Adenoviridae/genetics
  • Animals, Newborn
  • Cytoskeletal Proteins/analysis
  • Gene Expression
  • Genetic Therapy/methods
  • Genetic Vectors/administration & dosage
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Membrane Glycoproteins/analysis
  • Mice, Inbred mdx
  • Muscle, Skeletal/chemistry
  • Muscular Dystrophy, Animal/diagnosis
Suggested Control Mice
  • Wild-type littermates
  • Cardiovascular
  • Developmental Biology
  • Models for Human Disease
  • Neurobiology
  • Musculoskeletal
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)

Colony and Husbandry Information

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

Colony Surveillance Program and Current Health Reports

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

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

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.

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 $)
*Shipping & Handling not included*
Units Notes
000425-MU-EMBRYO Cryo-preserved embryos $1,038.00 / Non-Profit Aliquot Approximate quantity2 : 20-40 embryos / aliquot
000425-MU-SPERM Cryo-preserved spermatozoa $437.00 / Non-Profit Aliquot Approximate quantity3
000425-MU-RESUS Litter recovered from cryo-archive $2,016.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.

1 The distribution fee covers the expense of rederiving mice from a live mouse; 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 contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 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.

4 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.

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.