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B6N.Cg-Tg(Prnp-TARDBP*Q331K)109Dwc/J

Cat. No.: CEMM-07250384

Common Name: Prp-TDP-43Q331K-low line 109
Prp-TDP-43Q331K-low transgenic line 109 mice have expression of a myc-tagged, human TAR DNA binding protein carrying the ALS-linked Q331K mutation (huTDP-43*Q331K) directed to brain and spinal cord by the mouse prion protein promoter. Along with the moderate huTDP-43*Q331K overexpressing founder line (Prp-TDP-43Q331K [~1.5x] mice;), these Prp-TDP-43Q331K-low transgenic mice may be useful in studying motor dysfunction in the neurodegenerative disorder amyotrophic lateral sclerosis (ALS; Lou Gehrig's Disease).
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Status Live Mouse
Frozen Embryo
Age 4 weeks
12 weeks
Customized Age
Sex Male
Female
GENETICS
Allele Symbol
Tg(Prnp-TARDBP*Q331K)109Dwc
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Allele Name
transgene insertion 109
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Allele Attributes
Inserted expressed sequence; Humanized sequence
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Gene Symbol
Tg(Prnp-TARDBP*Q331K)109Dwc
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Gene Name
transgene insertion 109
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Chromosome
UN
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Expressed Genes
TARDBP, TAR DNA binding protein, human; myc tag
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MGI Accession ID
5463115
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Strain of Origin
C57BL/6 x C3H
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Molecular Note
A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was modified to have both an N-terminal myc tag and the glutamine to lysine substitution at amino acid 331 associated with familial ALS (huTDP-43*Q331K). This huTDP-43*Q331K cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (Prnp) gene. The resulting Prnp-TDP-43*Q331K transgene was flanked with loxP sites. Founder line 109 was identified with approximately the same total TDP-43 (slightly less huTDP-43*Q331K) expression levels in spinal cord as compared to endogenous TDP-43 in non-transgenic mice.
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HUSBANDRY
Suggested Controls
C57BL/6NJ Noncarrier
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Breeding Considerations
When maintaining a live colony, hemizygous mice may be bred with wildtype (non-carrier) mice from the colony or with C57BL/6NJ inbred mice. The donating investigator has not attempted to generate homozygous mice to date.
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Breeding Strategy
Noncarrier x Hemizygote; Hemizygote x Noncarrier
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For Research Use Only.
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