Name/
Symbol
|
Strain Name
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Transgene/
Promoter and Regulatory Elements
|
Genetic Background
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Behavioral
Phenotype
|
Neurological
Characteristics
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Patents/
Availability
|
Primary
Citation
|
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hTDP-43-ΔNLS and
hTDP-43-WT
Symbol: TDP-43
Posted 02/03/11
|
|
Tetracycline promoter from pTetSplice with two exons, one intron, and the original
39 UTR of the moPrP.XhoI vector (Jankowsky
et al., 2005).
|
Origin: C57BL/6J-C3HeJ. Special husbandry: maintained 28 days on Doxycycline to
inhibit transgene exp. then switched to standard chow to induce hTDP-43 expression.
|
Motor impairment observed in tTA/TDP-ΔNLS mice.
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hTDP-43-ΔNLS mice express hTDP-43 with a defective nuclear localization signal
in forebrain and changes in gene expression in cortical neurons.
|
Contact: Virginia Lee
|
Igaz et al., 2011
|
|
VCP R155H/+ KI
Symbol: TDP-43
Posted 02/03/11
|
|
Mouse VCP fragment/7.9kb upsteam seq and 2.1 downstream seq/R to H mutation at position
155/129/SvEv mice.
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Origin: C57BL/6; Backcross: N6; Generation: >10.
|
Progressive muscle weakness (6 months), vacuoles and inclusions in muscle fibers,
Paget like bone changes, typical brain pathology of IBMPFD.
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Increased TDP-43 and ubiquitin-positive inclusions in frontal cortex.
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Contact: Virginia E. Kimonis
|
Badadani et al., 2010
|
|
TDP-43Prp
Symbol: TDP-43
Posted 12/7/10
|
|
Full-length hTDP-43 cDNA/MoPr promoter.
|
Origin: C57BL/6; Background: C57BL/6.
|
Gait abnormalities, early lethality.
|
hTDP-43 caused downregulation of mouse TDP-43 RNA and protein. Also caused TDP-43
phosphorylation.
|
Contact: Dr. Jada Lewis
Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224
|
Xu et al., 2010
|
|
VCP R155H and VCP A232E
Symbol: VCP
Posted 12/7/10
|
|
Disease mutations R155H and A232E of human VCP (Valosin-containing protein) inserted
into cDNA/SJL pronuclei.
|
Origin: C57/B6; Background: C57/B6 F2.
|
Abnormal behavior, progressive muscle weakness, vertebrae and femur lesions.
|
Brain shows widespread TDP-43 pathology.
|
Contact: J. Paul Taylor
Developmental Neurobiology, MS 343, D-4026, St. Jude Children’s Research Hospital,
262 Danny Thomas Place, Memphis, TN 38105-3678
|
Custer et al., 2010
|
|
CaMKII-TDP-43
Symbol: TDP-43 OE
Posted 12/7/10
|
|
Full-length moTDP-43 DNA cloned into EcoRV site of pNN265/NotI frag isolated/cloned
into vector pMM403/CaMKII promoter/11.2kbsfil frag. purified/FVB/N embryos.
|
Origin: FVB/N; Generation: F8.
|
Learning deficits, progressive motor dysfunction and hippocampal atrophy.
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TDP-43 OE in hippocampus, cortex, and striatum leads to formation of TDP-43+, ubiquitin+
NCIs, and neurodegeneration.
|
Contact: Che-Kun James Shen
Institute of Molecular Biology, Academia Sinica, Taipei 115, Taiwan
|
Wu et al., 2010
|
|
TardbpGt(RB030)Byg
Symbol: TDP-43
Updated 02/03/11
|
|
Tardbp gene targeted with vector pGT11xf/ inserted within intron 2 at bp 1226/ES
cells RB030.
|
Origin: C57BL/6/129; Backcross: N5; C57BL/6
|
Homozygous are embryonic lethal. Het show motor disturbance, muscle weakness with
decreased forelimb grip strength.
|
No evidence of pathologic changes in motor neurons.
|
Contact: B.C. Kraemer
Geriatrics Research Education and Clinical Center, Veterans Affairs
Puget Sound Health Care System, Seattle, WA 98108
|
Kraemer et al., 2010
|
|
Wobbler
Symbol: Vps54
Posted 11/10/10
|
B6.B-Vps54wr/J.
|
Spontaneous mutation.
|
Origin: C57BL/Fa; Generation: N8+ (07-SEP-10).
|
Progressive locomotor impairment.
|
Motor neuron and muscular degeneration. Mice undergo TDP-43 and ubiquitin changes
characteristic of sporadic ALS.
Dennis and Citron, 2009
|
The Jackson Lab,
under development, stock #009680.
|
Falconer,
1956
|
|
Thy-1-hTDP-43
Symbol: TDP-43
Posted 10/11/10
|
|
Human TDP-43 cDNA/ mouse Thy1.2 promotor/ C57BL/6;SJL hybrid mouse embryos.
|
Origin: C57BL/6; SJL; Background: C57BL/6; Generation: F3N2.
|
Group W1 with the highest transgene copy die within three weeks of age. Group W2
and W3 exhibit less growth retardation and grow to adulthood.
|
Male (day 14-18) mice develop severe tremor, abnormal reflex of hindlimbs and gait
abnormalities. Female develop fine tremor only after three months of age.
|
Philip C. Wong
|
Shan et al., 2010
|
|
Conditional Tardbp-KO
Symbol: TDP-43
Posted 10/11/10
|
|
Conditional deletion of exon 3 of Tardbp.
|
Origin: V26.2 C57BL/6j ES cells; injected into albino C57BL/6J; Background: C57BL/6;
Generation: F3, N3.
|
Homozygous mice fertile. Postnatal deletion of Tardbp caused dramatic loss of body
fat followed by rapid death.
|
N/A
|
Philip C. Wong
|
Chiang et al., 2010
|
Tardbp+/-
Line: RRB030
Symbol: TDP-43
Posted 09/01/10
|
|
Genetrap/β-geo fusion protein/loss of functional domains of native protein.
|
Origin: 129P2/OlaHsd; Background: C57Bl/6; Generation: F6; Breeding: het to wt or
het; homozygous are embryonic lethal E3.5-E8.5.
|
Tardbp+/- mice are viable, fertile, normal in size and display no overt
phenotype.
|
LacZ staining in adult : Tardbp+/- by E12.5 shows widespread expression
in regions of CNS.
|
Contact Gang.Yu@UTSouthwestern.edu.
|
Sephton et al., 2010
|
|
TDP-43WT
Symbol: TDP-43
Posted 03/09/10
|
|
TARDBP cloned into mTUB expression vector/mouse Thy-1 promoter.
|
Origin: C56Bl6/J; Generation ~F4
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Abnormal limb reflex in highest expressing homozygous mice (14-day).
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Degeneration of spinal and cortical motor neurons and non-motor cortical neurons.
|
Samir Kumar-Singh
TAR4 (high Tg expressor) and TAR6 (low Tg expressor).
|
Wils et al., 2010
|
|
PrP-TDP43A315T
Symbol: TDP-43
Posted 03/09/10
|
B6;CB-Tg(Prnp-TARDBP*A315T)95Balo/J
|
Full-length hTARDBP/A315T inserted between exon 2-3/moPrP/founder line 95.
|
Origin: C57BL/6J x CBA.
|
Viable, fertile; hemizygous mice develop gait disorder.
|
Mice develop a progressive and fatal neurodegenerative disease reminiscent of both
ALS and FTLD.
|
The Jackson Lab,
under development, stock #010700. Use by companies or for-profit entities requires
a license prior to shipping.
|
Wegorzewska et al.,
2009
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