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Items: 1 to 20 of 184

1.

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.

Xu YF, Gendron TF, Zhang YJ, Lin WL, D'Alton S, Sheng H, Casey MC, Tong J, Knight J, Yu X, Rademakers R, Boylan K, Hutton M, McGowan E, Dickson DW, Lewis J, Petrucelli L.

J Neurosci. 2010 Aug 11;30(32):10851-9. doi: 10.1523/JNEUROSCI.1630-10.2010.

2.

Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.

Xu YF, Zhang YJ, Lin WL, Cao X, Stetler C, Dickson DW, Lewis J, Petrucelli L.

Mol Neurodegener. 2011 Oct 26;6:73. doi: 10.1186/1750-1326-6-73.

3.

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.

Igaz LM, Kwong LK, Lee EB, Chen-Plotkin A, Swanson E, Unger T, Malunda J, Xu Y, Winton MJ, Trojanowski JQ, Lee VM.

J Clin Invest. 2011 Feb;121(2):726-38. doi: 10.1172/JCI44867. Epub 2011 Jan 4.

4.

The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43.

Xu YF, Prudencio M, Hubbard JM, Tong J, Whitelaw EC, Jansen-West K, Stetler C, Cao X, Song J, Zhang YJ.

PLoS One. 2013 Jul 26;8(7):e69864. doi: 10.1371/journal.pone.0069864. Print 2013.

5.

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.

Walker AK, Spiller KJ, Ge G, Zheng A, Xu Y, Zhou M, Tripathy K, Kwong LK, Trojanowski JQ, Lee VM.

Acta Neuropathol. 2015 Nov;130(5):643-60. doi: 10.1007/s00401-015-1460-x. Epub 2015 Jul 22.

PMID:
26197969
6.

Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.

Janssens J, Wils H, Kleinberger G, Joris G, Cuijt I, Ceuterick-de Groote C, Van Broeckhoven C, Kumar-Singh S.

Mol Neurobiol. 2013 Aug;48(1):22-35. doi: 10.1007/s12035-013-8427-5. Epub 2013 Mar 10.

7.

Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.

Swarup V, Phaneuf D, Bareil C, Robertson J, Rouleau GA, Kriz J, Julien JP.

Brain. 2011 Sep;134(Pt 9):2610-26. doi: 10.1093/brain/awr159. Epub 2011 Jul 13.

8.

Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.

Stribl C, Samara A, Trümbach D, Peis R, Neumann M, Fuchs H, Gailus-Durner V, Hrabě de Angelis M, Rathkolb B, Wolf E, Beckers J, Horsch M, Neff F, Kremmer E, Koob S, Reichert AS, Hans W, Rozman J, Klingenspor M, Aichler M, Walch AK, Becker L, Klopstock T, Glasl L, Hölter SM, Wurst W, Floss T.

J Biol Chem. 2014 Apr 11;289(15):10769-84. doi: 10.1074/jbc.M113.515940. Epub 2014 Feb 10.

9.

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration.

Wils H, Kleinberger G, Janssens J, Pereson S, Joris G, Cuijt I, Smits V, Ceuterick-de Groote C, Van Broeckhoven C, Kumar-Singh S.

Proc Natl Acad Sci U S A. 2010 Feb 23;107(8):3858-63. doi: 10.1073/pnas.0912417107. Epub 2010 Feb 3.

10.
11.

Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.

Mitchell JC, Constable R, So E, Vance C, Scotter E, Glover L, Hortobagyi T, Arnold ES, Ling SC, McAlonis M, Da Cruz S, Polymenidou M, Tessarolo L, Cleveland DW, Shaw CE.

Acta Neuropathol Commun. 2015 Jun 25;3:36. doi: 10.1186/s40478-015-0212-4.

12.

Neuronal sensitivity to TDP-43 overexpression is dependent on timing of induction.

Cannon A, Yang B, Knight J, Farnham IM, Zhang Y, Wuertzer CA, D'Alton S, Lin WL, Castanedes-Casey M, Rousseau L, Scott B, Jurasic M, Howard J, Yu X, Bailey R, Sarkisian MR, Dickson DW, Petrucelli L, Lewis J.

Acta Neuropathol. 2012 Jun;123(6):807-23. doi: 10.1007/s00401-012-0979-3. Epub 2012 Apr 27.

13.

The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth.

Fallini C, Bassell GJ, Rossoll W.

Hum Mol Genet. 2012 Aug 15;21(16):3703-18. doi: 10.1093/hmg/dds205. Epub 2012 May 28.

14.

Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration.

Nishimura AL, Zupunski V, Troakes C, Kathe C, Fratta P, Howell M, Gallo JM, Hortobágyi T, Shaw CE, Rogelj B.

Brain. 2010 Jun;133(Pt 6):1763-71. doi: 10.1093/brain/awq111. Epub 2010 May 14.

15.
16.

C57BL/6J congenic Prp-TDP43A315T mice develop progressive neurodegeneration in the myenteric plexus of the colon without exhibiting key features of ALS.

Hatzipetros T, Bogdanik LP, Tassinari VR, Kidd JD, Moreno AJ, Davis C, Osborne M, Austin A, Vieira FG, Lutz C, Perrin S.

Brain Res. 2014 Oct 10;1584:59-72. doi: 10.1016/j.brainres.2013.10.013. Epub 2013 Oct 18.

PMID:
24141148
17.

Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.

Tsai KJ, Yang CH, Fang YH, Cho KH, Chien WL, Wang WT, Wu TW, Lin CP, Fu WM, Shen CK.

J Exp Med. 2010 Aug 2;207(8):1661-73. doi: 10.1084/jem.20092164. Epub 2010 Jul 26.

18.

Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord.

Braak H, Ludolph A, Thal DR, Del Tredici K.

Acta Neuropathol. 2010 Jul;120(1):67-74. doi: 10.1007/s00401-010-0683-0. Epub 2010 Apr 9.

PMID:
20379728
19.

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.

Wegorzewska I, Bell S, Cairns NJ, Miller TM, Baloh RH.

Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18809-14. doi: 10.1073/pnas.0908767106. Epub 2009 Oct 15.

20.

TARDBP pathogenic mutations increase cytoplasmic translocation of TDP-43 and cause reduction of endoplasmic reticulum Ca²⁺ signaling in motor neurons.

Mutihac R, Alegre-Abarrategui J, Gordon D, Farrimond L, Yamasaki-Mann M, Talbot K, Wade-Martins R.

Neurobiol Dis. 2015 Mar;75:64-77. doi: 10.1016/j.nbd.2014.12.010. Epub 2014 Dec 17.

PMID:
25526708
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