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B6;129S6-Gt(ROSA)26Sortm1(TARDBP*M337V/Ypet)Tlbt/J
Cat. No.: CEMM-07250718
Common Name: TDP-43M337V BAC knock-in
TDP-43M337V BAC allele is a Gt(ROSA)26Sor knock-in of a loxP-flanked, full-length human TARDBP genomic locus with M337V mutation in exon 6 and C-terminal fluorescent tag. Prior to Cre recombinase exposure, TDP-43M337V BAC mice have stable, single-copy, low-level expression of TDP-43M337V-Ypet that recapitulates many of the key features of ALS/FTD (amyotrophic lateral sclerosis [Lou Gehrig's disease] / frontotemporal dementia [frontotemporal lobar degeneration]), including progressive motor deficits in conjunction with neuromuscular junction abnormalities and reduced survival.
Inquiry
| Status | Live Mouse Frozen Embryo |
| Age | 4 weeks 12 weeks Customized Age |
| Sex | Male Female |
| GENETICS | |
|---|---|
| Allele Symbol |
Gt(ROSA)26Sortm1(TARDBP*M337V/Ypet)Tlbt
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| Allele Name |
targeted mutation 1
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| Allele Attributes |
Conditional ready (e.g. floxed); Reporter; Inserted expressed sequence; Humanized sequence
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| Gene Symbol |
Gt(ROSA)26Sor
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| Gene Name |
gene trap ROSA 26
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| Chromosome |
6
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| Expressed Genes |
TARDBP, TAR DNA binding protein, human; YFP, Yellow Fluorescent Protein, jellyfish
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| MGI Accession ID | show more close |
| Site of Expression |
When these mutant mice are bred to mice that express Cre recombinase, the resulting offspring will express YFP (revealed by immunostaining via antibody directed against GFP) in the Cre-expressing tissues.
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| Strain of Origin |
(C57BL/6J x 129S6/SvEvTac)F1
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| Molecular Note |
The human TARDBP from BAC RP11-829B14 was modified by site-directed mutagenesis to Create the M337V missense mutation in exon 6 (an A-to-G base change (A1009G) encoding a methionine-to-valine substitution at amino acid position 337). The final construct includes the TDP-43-M337V genomic DNA (44,886 bp) with a Ypet ORF cDNA sequence (717 bp; described below) fused to the 5' end of TARDBP exon 6, as well as 9.2 kbp of the original BAC backbone sequences. The TDP-43-M337V-Ypet BAC sequences are flanked with loxP sites. Embryonic stem (ES) cells with a pre-integrated Gt(ROSA)26Sor locus (splice acceptor::FRT::PGK promoter-attP-hygromycin resistance gene-pA::FRT::attP between exons 1 and 2) were re-transfected with the TDP-43-M337V-Ypet BAC sequence and a PhiC31 vector for integrase-mediated cassette exchange. The Ypet cDNA sequence is a codon-improved YFP variant YFP3 (with F46L, I47L, S208F, V224L, H231E, D234N mutations) further modified to include several Venus mutations (F64L, R79K, M153T, V163A, S175G), resulting in the YFP for energy transfer (YPet) variant.
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| HUSBANDRY | |
|---|---|
| Suggested Controls |
C57BL/6J
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| Breeding Considerations |
Homozygous and heterozygous mice are viable past one year of age and fertile, with no apparent deficits in fertility or litter size compared to controls. When maintaining a live colony, heterozygous mice may be bred together, to wildtype mice from the colony or to C57BL/6J inbred mice. Alternatively, homozygous mice may be bred together.
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For Research Use Only.
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