These transgenic animals express the mouse Serpina3n gene under the regulatory control of the human skeletal muscle-specific ACTA1/SKA promoter. When crossed to muscular dystrophy mouse models, the dystrophic disease phenotype is rescued.
Jeffery D. Molkentin, Cincinnati Children's Hospital
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence) |
Mouse models of muscular dystrophy show a compensatory increase in the protein encoded by the Serpina3n (serine (or cysteine) peptidase inhibitor, clade A, member 3N) gene after acute muscle tissue injury.
These healthy transgenic mice express mouse Serpina3n under the regulatory control of the human ACTA1 (actin, alpha 1, skeletal muscle; also known as SKA) promoter. Expression has been demonstrated in fast skeletal muscles including the quadriceps, tibialis anterior, gastrocnemius, and flexor digitorum brevis. More slow/oxidative fiber-type muscles, such as the soleus and diaphragm, show lower levels of protein over-expression and the heart shows no transgene expression. Muscle histology appears normal at both 6 weeks and 10 months of age.
The transgene dramatically reduces skeletal muscle pathogenesis in both Sgcd (δ-sarcoglycan deficient; e.g. Stock No. 004582) and Dmdmdx (dystrophin deficient) mouse models of muscular dystrophy. Muscle degeneration and fibrosis are mitigated, creatine kinase serum levels are reduced, running capacity is restored, and in vivo muscle membrane leakiness is reduced.
Mouse Serpina3n followed by a polyA tail is overexpressed under the control of a modified human ACTA1/SKA promoter (likely including the -2000 to +239 bp region driving enhanced expression in skeletal muscles). This strain was created and maintained on an FVB genetic background by the donating lab.
Expressed Gene | Serpina3n, serine (or cysteine) peptidase inhibitor, clade A, member 3N, mouse, laboratory |
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Site of Expression |
Allele Name | transgene insertion 1, Jeffery Molkentin |
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Allele Type | Transgenic (Inserted expressed sequence) |
Allele Synonym(s) | Serpina3n TG |
Gene Symbol and Name | Tg(ACTA1-Serpina3n)1Jmol, transgene insertion 1, Jeffery Molkentin |
Gene Synonym(s) | |
Promoter | ACTA1, actin, alpha 1, skeletal muscle, human |
Expressed Gene | Serpina3n, serine (or cysteine) peptidase inhibitor, clade A, member 3N, mouse, laboratory |
Strain of Origin | FVB/N |
Chromosome | UN |
Molecular Note | The transgenic construct contains mouse Serpina3n followed by a polyA tail under the control of a modified human ACTA1/SKA promoter. |
Hemizygotes are viable and fertile.
When using the FVB/N-Tg(ACTA1-Serpina3n)1Jmol/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #028792 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrier for Tg(ACTA1-Serpina3n)1Jmol |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Serpina3n)1Jmol/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Serpina3n)1Jmol/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Serpina3n)1Jmol/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | FVB/N-Tg(ACTA1-Serpina3n)1Jmol/J Frozen Embryo | $3373.50 |
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