Prp-TDP-43Wild-Type transgenic mice have expression of a myc-tagged, human wildtype TAR DNA binding protein (huTDP-43) directed to brain and spinal cord by the mouse prion protein promoter. Along with mice expressing the ALS-linked Q331K mutation (Prp-TDP-43Q331K-low mice (Stock No. 017930) and Prp-TDP-43Q331K [~1.5x] mice (Stock No. 017933)), these mice may be useful in studying the neurodegenerative disorder amyotrophic lateral sclerosis (ALS; Lou Gehrig's Disease).
Dr. Don Cleveland, Ludwig Institute for Cancer Res. (UCSD)
Prp-TDP-43Wild-Type transgenic mice express a myc-tagged, human wildtype TAR DNA binding protein (huTDP-43) cDNA sequence under the direction of the mouse prion protein promoter (PrP). As expected for the PrP promoter, transgene expression is confined primarily to central nervous system (brain and spinal cord), with very low to no expression in other tested tissues (testis not examined). Anti-myc antibody staining shows huTDP-43 accumulation in the nuclei of neurons as well as glial cells of the spinal cord and brain, with a corresponding downregulation of endogenous mouse TDP-43. The transgene is flanked by loxP sites, allowing it to be removed by introduction of Cre recombinase if desired. The phenotype below describes Prp-TDP-43Wild-Type transgenic mice from founder line 96.
The Prp-TDP-43Wild-Type mice have moderate overexpression levels in total TDP-43 mRNA/protein, with an ~1.5-fold increase in total TDP-43 expression (huTDP-43-WT levels approximately the same) as compared to endogenous TDP-43 in non-transgenic mice. Prp-TDP-43Wild-Type mice do not develop adult-onset motor dysfunction and do not exhibit any loss of motor axons or neurons. These Prp-TDP-43Wild-Type mice are a control strain for similarly designed mice expressing the Q331K ALS-linked mutation (Prp-TDP-43Q331K-low mice (Stock No. 017930) and Prp-TDP-43Q331K [~1.5x] mice (Stock No. 017933)).
A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was modified to have an N-terminal myc tag. This huTDP-43-WT 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 MoPrP.XhoI plasmid vector (ATCC#JHU-2). The resulting MoPrP.XhoI-mychuTDP-43-WT transgene was flanked with loxP sites, and then injected into the pronuclei of fertilized C57BL6/C3H hybrid eggs, which were implanted into pseudopregnant female mice. Transgenic founder mice were bred to C57BL/6 mice, and founder line 96 was identified with ~1.5-fold greater total TDP-43 (approximately the same huTDP-43-WT) expression levels in spinal cord as compared to endogenous TDP-43 in non-transgenic mice. These Prp-TDP-43Wild-Type transgenic mice were bred with C57BL/6NCrl females for at least eight generations prior to sending to The Jackson Laboratory Repository. Upon arrival, transgenic mice were bred to C57BL/6NJ inbred mice (Stock No. 005304) for at least one generations to establish The Jackson Laboratory Repository colony.
|Expressed Gene||TARDBP, TAR DNA binding protein, human|
|Site of Expression|
|Allele Name||transgene insertion 96, Don W Cleveland|
|Allele Type||Transgenic (Humanized sequence, Inserted expressed sequence)|
|Gene Symbol and Name||Tg(Prnp-TARDBP)96Dwc, transgene insertion 96, Don W Cleveland|
|Promoter||Prnp, prion protein, mouse, laboratory|
|Expressed Gene||TARDBP, TAR DNA binding protein, human|
|Strain of Origin||C57BL/6 x C3H|
When maintaining a live colony, hemizygous mice may be bred with wildtype (noncarrier) mice from the colony or with C57BL/6NJ inbred mice (Stock No. 005304). The donating investigator has not attempted to generate homozygous mice to date (May 2012).
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