Updated 3 February 2011

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Name/ Symbol Strain Name Transgene/ Promoter and Regulatory Elements Genetic Background Behavioral  Phenotype Neurological  Characteristics Patents/  Availability Primary  Citation 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. 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. 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. Increased TDP-43 and ubiquitin-positive inclusions in frontal cortex. 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. 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 Abnormal limb reflex in highest expressing homozygous mice (14-day). 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