TDP43 transgenic mice express a full-length human TAR DNA binding protein (TARDBP) cDNA under the control of mouse prion protien promoter. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease) and frontotemporal lobar degeneration with ubiquitin aggregates.
Jeffrey L Elliott, The University of Texas Southwestern Medical Center
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
TDP43 transgenic mice express a full length human TAR DNA binding protein (TARDBP or TDP-43) cDNA inserted between exon 2 and exon 3 of the mouse prion protein (Prnp) gene. The transgene in founder line 4 was later found to have integrated on the X chromosome. Hemizygotes are viable and fertile, with mean survival of 109 days. TDP-43 is a ubiquinated protein localized to the nucleus of nerve cells. Accumulations of mutated TDP-43 are involved in the development of Amyotrophic lateral sclerosis (ALS). These mice express human TDP-43 in the spinal cord and skeletal muscle. They exhibit progressive neurodegeneration characterized by bilateral proximal weakness, spasticity, reduced spontaneous movements and progressive weight loss. Variable phenotype penetrance is observed and some animals remain asymptomatic at 6 months of age. These mice contain a nuclear localized, less truncated form of TDP-43 than that observed in TDP-43*A315T mice (Stock No. 016143). Disease progression is later than that observed in TDP-43*A315T mice.
A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene at two unique XhoI sites in the Mo-Prp.Xho plasmid vector (ATCC#JHU-2). The resulting transgene was microinjected into B6SJLF1 oocytes and mice from founder line 4, in which the transgene integrated on the X chromosome, were bred with CD1 mice to establish a colony. These mice were maintained on a mixed background. Upon arrival at The Jackson Laboratory, transgenic mice were bred with non-transgenic littermates to establish the colony.
Expressed Gene | TARDBP, TAR DNA binding protein, human |
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Site of Expression |
Allele Name | transgene insertion 4, Jeffrey L Elliott |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | WT TDP-43 line 4 |
Gene Symbol and Name | Tg(Prnp-TARDBP)4Jlel, transgene insertion 4, Jeffrey L Elliott |
Gene Synonym(s) | |
Promoter | Prnp, prion protein, mouse, laboratory |
Expressed Gene | TARDBP, TAR DNA binding protein, human |
Strain of Origin | (C57BL/6 x SJL)F1 |
Chromosome | UN |
Molecular Note | The mouse promoter drives neuronal expression of the wild-type human cDNA. Several lines were established (including 4, 13, and 21). |
Mutations Made By | Jeffrey Elliott, The University of Texas Southwestern Medical Center |
When maintaining a live colony, hemizygous mice may be bred to wild type from the colony. The transgene in this founder line was found to have integrated on the X chromosome. These mice have a mean survival time of 109 days and exhibit progressive neurodegeneration characterized by decreased motor function, however variable phenotype penetrance is observed and some animals remain asymptomatic at 6 months of age.
When using the STOCK Tg(Prnp-TARDBP)4Jlel/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #016144 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous females and Non Carrier Males for Tg(Prnp-TARDBP)4Jlel/ |
Frozen Mouse Embryo | STOCK Tg(Prnp-TARDBP)4Jlel/J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | STOCK Tg(Prnp-TARDBP)4Jlel/J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | STOCK Tg(Prnp-TARDBP)4Jlel/J Frozen Embryos | $3373.50 |
Frozen Mouse Embryo | STOCK Tg(Prnp-TARDBP)4Jlel/J Frozen Embryos | $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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