TDP-43PrP transgenic mice express a full length wild type human TARDBP cDNA under control of a Prnp promoter. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease).
Leonard Petrucelli, Mayo Clinic
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
TDP-43PrP transgenic mice express a full length wild type human TAR DNA binding protein (TARDBP or TDP-43) cDNA under control of the prion protein (Prnp) promoter. Hemizygotes are viable, fertile, and normal in size. TDP-43 is a ubiquinated protein localized to the nucleus of cells. Accumulations of TDP-43 are involved in the development of Amyotrophic lateral sclerosis (ALS). These mice express wildtype human TDP-43 primarily in the nuclei of neurons throughout much of the gray matter of the spinal cord and brain, including the hippocampus, striatum, brainstem and cortex. Homozygotes and hemizygous express TDP-43 in brain at 2.5 and 1.9-fold endogenous TDP-43 levels, respectively. Endogenous TDP-43 is down-regulated in response to transgene overexpression. Beginning at 14 days of age, homozygous TDP-43PrP are smaller than hemizygous littermates. By 21 days of age homozygotes develop body tremors, have difficulty walking, and develop an aberrant "swimming" gait. Between 1 and 2 months of age they become unable to right themselves. These also mice exhibit reactive gliosis, and axonal and myelin degeneration. These mice exhibit truncated TDP-43 and ubiquitin aggregates containing phosphorylated TDP-43. Hemizygous mice are indistinguishable from wildtype littermates. 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.
The TDP-43PrP transgene was designed with the human TARDBP gene driven by a mouse prion protein (Prnp) promoter. The transgene was microinjected into fertilized C57BL/6 oocytes. Mice from the highest expressing founder line were bred to C57BL/6 mice to establish a colony. Upon arrival at The Jackson Laboratory, transgenic mice were bred to C57BL/6J mice (Stock No. 000664).
Expressed Gene | TARDBP, TAR DNA binding protein, human |
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Site of Expression |
Allele Name | transgene insertion 3c, Leonard Petrucelli |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | TDP-43PrP; Tg(Prnp-TARDBP)3cLtrc |
Gene Symbol and Name | Tg(Prnp-TARDBP)3cPtrc, transgene insertion 3c, Leonard Petrucelli |
Gene Synonym(s) | |
Promoter | Prnp, prion protein, mouse, laboratory |
Expressed Gene | TARDBP, TAR DNA binding protein, human |
Strain of Origin | C57BL/6 |
Chromosome | UN |
Molecular Note | The TDP-43PrP transgene was designed with the human TAR DNA binding protein (TARDBP or TDP-43) gene driven by a mouse prion protein (Prnp) promoter. Line 3c was generated. |
Mutations Made By | Leonard Petrucelli, Mayo Clinic |
When maintaining a live colony, these mice may be bred as hemizygotes.
When using the TDP-43PrP mouse strain in a publication, please cite the originating article(s) and include JAX stock #016608 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrier for Tg(Prnp-TARDBP)3cLtrc |
Frozen Mouse Embryo | C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J Frozen Embryo | $3373.50 |
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