These mice carry a transgene containing a human skeletal actin (HSA1) promoter upstream of a bovine poly-(A) binding protein nuclear 1 (PABPN1) with 17 alanines. Mice recovered from cryopreserved stock exhibited a loss of expected phenotype (progressive muscle weakness as assessed by locomotor activity and grip strength). A replacement line was obtained from the Donating Investigator, FVB-Tg(ACTA1-PABPN1*A17)1Drub/DrubJ, Stock No. 016193.
Prof. David Rubinsztein, Cambridge Institute for Medical Research
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence) |
These mice carry a transgene containing a human skeletal actin (HSA1) promoter upstream of a bovine poly-(A) binding protein nuclear 1 (PABPN1) with 17 alanines. Mice recovered from cryopreserved stock exhibited a loss of expected phenotype (progressive muscle weakness as assessed by locomotor activity and grip strength). A replacement line was obtained from the Donating Investigator, FVB-Tg(ACTA1-PABPN1*A17)1Drub/DrubJ, Stock No. 016193.
A transgenic construct containing a human skeletal actin (HSA1) promoter upstream of a bovine poly-(A) binding protein nuclear 1 (PABPN1) with 17 alanines was injected into FVB donor eggs.
Expressed Gene | PABPN1, poly(A) binding protein nuclear 1, human |
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Site of Expression |
Allele Name | transgene insertion 1, David Rubinsztein |
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Allele Type | Transgenic (Inserted expressed sequence) |
Allele Synonym(s) | A17.1; A17-1 |
Gene Symbol and Name | Tg(ACTA1-PABPN1*A17)1Drub, transgene insertion 1, David Rubinsztein |
Gene Synonym(s) | |
Promoter | ACTA1, actin, alpha 1, skeletal muscle, human |
Expressed Gene | PABPN1, poly(A) binding protein nuclear 1, human |
Strain of Origin | FVB/N |
Chromosome | UN |
General Note | Of two lines descended from founders with this transgenic construct, line A7-1 has a more severe phenotype, correlated with higher transgene expression levels, than line A7-2. |
Molecular Note | The transgene contains the human skeletal actin promoter upstream of a mutant bovine cDNA with a series of CGC codons encoding a 17-amino acid polyalanine tract. |
When maintaining a live colony, these mice are bred as hemizygotes. Homozygotes of this strain have not been created.
When using the A17-1 mouse strain in a publication, please cite the originating article(s) and include JAX stock #006655 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-PABPN1*A17)1Drub |
Frozen Mouse Embryo | FVB-Tg(ACTA1-PABPN1*A17)1Drub/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB-Tg(ACTA1-PABPN1*A17)1Drub/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB-Tg(ACTA1-PABPN1*A17)1Drub/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | FVB-Tg(ACTA1-PABPN1*A17)1Drub/J Frozen Embryo | $3373.50 |
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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.
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