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.
Kevin Campbell, University of IowaRead More +
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.
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.
|Expressed Gene||SGCE, sarcoglycan epsilon, human|
|Site of Expression|
|Allele Name||targeted mutation 1, Kevin P Campbell|
|Allele Type||Targeted (Null/Knockout)|
|Allele Synonym(s)||KO/KO; Sgcanull; Sgcatm594Kcam; alphaSG-|
|Gene Symbol and Name||Sgca, sarcoglycan, alpha (dystrophin-associated glycoprotein)|
|Gene Synonym(s)||50DAG; ADL; Asg; DAG2; DMDA2; LGMD2D; SCARMD1; adhalin|
|Strain of Origin||(129X1/SvJ x 129S1/Sv)F1-Kitl<+>|
|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.|
|Allele Name||transgene insertion 1, Kevin Campbell|
|Allele Type||Transgenic (Humanized sequence, Inserted expressed sequence)|
|Gene Symbol and Name||Tg(Ckm-SGCE)1Kcam, transgene insertion 1, Kevin Campbell|
|Promoter||Ckm, creatine kinase, muscle, mouse, laboratory|
|Expressed Gene||SGCE, sarcoglycan epsilon, human|
|Strain of Origin||(C57BL/6 x SJL)F1|
|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.|
The donating investigator crosses homozygous Sgca targeted mutant/hemizygous SGCE transgenic males with homozygous Sgca targeted mutant females to maintain a colony.
|Please inquire about possible genotypes.|
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided,
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