Transgenic animals expressing mouse Ctss under the regulation of the skeletal muscle-specific human ACTA1 promoter begin developing pathologic features of muscular dystrophy around 2 months of age.
Jeffery D. Molkentin, Cincinnati Children's Hospital
Genetic Background | Generation |
---|---|
|
Allele Type |
---|
Transgenic (Inserted expressed sequence) |
Profiling of mRNA in humans with Duchenne muscular dystrophy (DMD) shows an increase in Ctss (cathepsin S) expression in injured skeletal muscle.
This transgenic strain places mouse Ctss cDNA under the control of a modified human ACTA1 (actin, alpha 1, skeletal muscle) promoter. Expression has been demonstrated in the quadriceps, tibialis anterior, gastrocnemius, and flexor digitorum brevis. Expression is uniform in multiple skeletal muscles that are predominantly fast-twitch, although lower levels of expression are observed in the soleus with its greater slow-fiber content. The heart shows no expression.
Hemizygous mice show greater muscle Ctss proteolytic activity that is associated with the induction of increased myofiber necrosis, muscle histopathology, and functional deficits that are reminiscent of muscular dystrophy. Histological analysis of skeletal muscle from hemizygous mice shows disease at 2 months of age which is characterized by accumulation of central nuclei in presumed regenerating myofibers, fatty tissue replacement and fibrosis, all features of muscular dystrophy. Similar tissue histopathology, with increased quantitative indexes of disease, are observed in skeletal muscle at 10 months of age. A decline in functional performance is correlated with the pathogenic changes, as assessed by treadmill running at both 2 and 4 months of age.
Hemizygous males may be infertile, thus hemizygous female x non-carrier male crosses are recommended to maintain a colony.
This transgene places the mouse Ctss cDNA under the control of a modified human ACTA1 promoter. This mutation was created and maintained on an FVB genetic background by the donating laboratory.
Expressed Gene | Ctss, cathepsin S, mouse, laboratory |
---|---|
Site of Expression | Skeletal muscle. |
Allele Name | transgene insertion 1, Jeffery Molkentin |
---|---|
Allele Type | Transgenic (Inserted expressed sequence) |
Allele Synonym(s) | Ctss Tg |
Gene Symbol and Name | Tg(ACTA1-Ctss)1Jmol, transgene insertion 1, Jeffery Molkentin |
Gene Synonym(s) | |
Promoter | ACTA1, actin, alpha 1, skeletal muscle, human |
Expressed Gene | Ctss, cathepsin S, mouse, laboratory |
Site of Expression | Skeletal muscle. |
Strain of Origin | FVB/N |
Chromosome | UN |
Molecular Note | The transgene consists of a Ctss cDNA sequence under the control of a modified human ACTA1 promoter, which is active in skeletal muscle. Western blot analysis confirms overexpression in multiple skeletal muscles that are predominantly fast-twitch, with lower levels of expression in soleus (slow-twitch). Founder line 1 is the higher expressing transgenic line. |
The donating lab maintained this strain through hemizygous female x non-carrier male crosses. Hemizygous males may be infertile. Hemizygous females are reported to breed normally between 2 and 8 months of age.
When using the Ctss TG mouse strain in a publication, please cite the originating article(s) and include JAX stock #028997 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Hemizygous or non carrier for Tg(ACTA1-Ctss)1Jmol |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Ctss)1Jmol/J | $2595.00 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Ctss)1Jmol/J | $2595.00 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Ctss)1Jmol/J | $3373.50 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Ctss)1Jmol/J | $3373.50 |
Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.
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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
What information were you hoping to find through your search?
How easy was it to find what you were looking for?
We may wish to follow up with you. Enter your email if you are happy for us to connect and reachout to you with more questions.
Please Enter a Valid Email Address
Thank you for sharing your feedback! We are working on improving the JAX Mice search. Come back soon for exciting changes.