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Items: 1 to 20 of 241

1.

ALS/FTLD-linked TDP-43 regulates neurite morphology and cell survival in differentiated neurons.

Han JH, Yu TH, Ryu HH, Jun MH, Ban BK, Jang DJ, Lee JA.

Exp Cell Res. 2013 Aug 1;319(13):1998-2005. doi: 10.1016/j.yexcr.2013.05.025. Epub 2013 Jun 4.

PMID:
23742895
2.

Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.

Ke YD, van Hummel A, Stevens CH, Gladbach A, Ippati S, Bi M, Lee WS, Krüger S, van der Hoven J, Volkerling A, Bongers A, Halliday G, Haass NK, Kiernan M, Delerue F, Ittner LM.

Acta Neuropathol. 2015 Nov;130(5):661-78. doi: 10.1007/s00401-015-1486-0. Epub 2015 Oct 5.

PMID:
26437864
3.

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.

4.

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.

5.

Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.

Barmada SJ, Skibinski G, Korb E, Rao EJ, Wu JY, Finkbeiner S.

J Neurosci. 2010 Jan 13;30(2):639-49. doi: 10.1523/JNEUROSCI.4988-09.2010.

6.

Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

Riku Y, Watanabe H, Yoshida M, Tatsumi S, Mimuro M, Iwasaki Y, Katsuno M, Iguchi Y, Masuda M, Senda J, Ishigaki S, Udagawa T, Sobue G.

JAMA Neurol. 2014 Feb;71(2):172-9. doi: 10.1001/jamaneurol.2013.5489.

PMID:
24378564
7.

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.

8.

Human TDP-43 and FUS selectively affect motor neuron maturation and survival in a murine cell model of ALS by non-cell-autonomous mechanisms.

Wächter N, Storch A, Hermann A.

Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(7-8):431-41. doi: 10.3109/21678421.2015.1055275. Epub 2015 Sep 7.

PMID:
26174443
9.

[Clinical and pathological spectrum of TDP-43 associated ALS].

Onodera O, Yokoseki A, Tan CF, Ishihara T, Nishiira Y, Toyoshima Y, Kakita A, Nishizawa M, Takahashi H.

Rinsho Shinkeigaku. 2010 Nov;50(11):940-2. Review. Japanese.

PMID:
21921519
10.

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.

11.

Progranulin and TDP-43: mechanistic links and future directions.

Kumar-Singh S.

J Mol Neurosci. 2011 Nov;45(3):561-73. doi: 10.1007/s12031-011-9625-0. Epub 2011 Aug 24. Review.

12.

Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS.

Ito D, Suzuki N.

Neurology. 2011 Oct 25;77(17):1636-43. doi: 10.1212/WNL.0b013e3182343365. Epub 2011 Sep 28. Review.

13.

The role of TDP-43 in the pathogenesis of ALS and FTLD.

Baralle M, Buratti E, Baralle FE.

Biochem Soc Trans. 2013 Dec;41(6):1536-40. doi: 10.1042/BST20130186. Review.

PMID:
24256250
14.

Disease-associated mutations of TDP-43 promote turnover of the protein through the proteasomal pathway.

Araki W, Minegishi S, Motoki K, Kume H, Hohjoh H, Araki YM, Tamaoka A.

Mol Neurobiol. 2014 Dec;50(3):1049-58. doi: 10.1007/s12035-014-8644-6. Epub 2014 Jan 30.

PMID:
24477737
15.

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
16.

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.

17.

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.

18.

The frontotemporal syndromes of ALS. Clinicopathological correlates.

Strong MJ, Yang W.

J Mol Neurosci. 2011 Nov;45(3):648-55. doi: 10.1007/s12031-011-9609-0. Epub 2011 Aug 2. Review.

PMID:
21809041
19.

TDP-43-induced death is associated with altered regulation of BIM and Bcl-xL and attenuated by caspase-mediated TDP-43 cleavage.

Suzuki H, Lee K, Matsuoka M.

J Biol Chem. 2011 Apr 15;286(15):13171-83. doi: 10.1074/jbc.M110.197483. Epub 2011 Feb 21.

20.

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons.

Wang W, Li L, Lin WL, Dickson DW, Petrucelli L, Zhang T, Wang X.

Hum Mol Genet. 2013 Dec 1;22(23):4706-19. doi: 10.1093/hmg/ddt319. Epub 2013 Jul 4.

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