MMRRC:000425-MU Detail Page

Strain Name: B6.129-Sgca^tm1Kcam/Mmmh

Stock Number: 000425-MU

Other Names:

alpha-sarcoglycan knock-out

Gene Details:

Allele Symbol: Sgca^tm575Kcam
Name: sarcoglycan, alpha (dystrophin-associated glycoprotein) / (unnamed provisional allele)
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:
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:49992)

elevated serum levels of creatine kinase activity in 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

Strain Type:

Founder genetic background: 129xC57BL/6

Strain genetic background: C57BL/6J

Strain Development:

Research Applications

Cardiovascular
Developmental Biology
Models for Human Disease
Neurobiology
Musculoskeletal

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:

Please complete the MMRRC Conditions of Use (COU) and provide it at the time your order is placed. Note: The preceding link is to the COU text only; the COU user form will be provided after order submission.

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-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

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 (207) 288-6009 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to information.

MMRRC Contact Information Home Page Feedback
The MMRRC is a collaborative effort, funded by grants from the National Center for Research Resources , NIH, DHHS.

Web forms on this site assume JavaScript enabled for Netscape 6.x, Microsoft Internet Explorer 5.5 and later versions; forms will not function as expected with earlier versions or when JavaScript is turned off.

Last Modified: June 23, 2010

Strain Detail Sheet

Strain Name: B6.129-Sgca^tm1Kcam/Mmmh

Stock Number: 000425-MU

Other Names:

Gene Details:

Strain Description

Sgca^tm1Kcam/Sgca^tm1Kcam

Research Applications

Strain Origin

Special Considerations

Health Status Report

Order Request Information

Availability Level:

Conditions of Distribution:

Fees: *

Control Mice:

Strain Detail Sheet

Strain Name: B6.129-Sgcatm1Kcam/Mmmh

Stock Number: 000425-MU

Other Names:

Gene Details:

Strain Description

Sgcatm1Kcam/Sgcatm1Kcam

Research Applications

Strain Origin

Special Considerations

Health Status Report

Order Request Information

Availability Level:

Conditions of Distribution:

Fees: *

Control Mice:

Strain Name: B6.129-Sgca^tm1Kcam/Mmmh

Sgca^tm1Kcam/Sgca^tm1Kcam