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Items: 1 to 20 of 118

1.

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

Arnold ES, Ling SC, Huelga SC, Lagier-Tourenne C, Polymenidou M, Ditsworth D, Kordasiewicz HB, McAlonis-Downes M, Platoshyn O, Parone PA, Da Cruz S, Clutario KM, Swing D, Tessarollo L, Marsala M, Shaw CE, Yeo GW, Cleveland DW.

Proc Natl Acad Sci U S A. 2013 Feb 19;110(8):E736-45. doi: 10.1073/pnas.1222809110. Epub 2013 Feb 4.

2.

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.

3.

Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies.

Cascella R, Capitini C, Fani G, Dobson CM, Cecchi C, Chiti F.

J Biol Chem. 2016 Sep 9;291(37):19437-48. doi: 10.1074/jbc.M116.737726. Epub 2016 Jul 21.

4.

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.

5.

Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.

Highley JR, Kirby J, Jansweijer JA, Webb PS, Hewamadduma CA, Heath PR, Higginbottom A, Raman R, Ferraiuolo L, Cooper-Knock J, McDermott CJ, Wharton SB, Shaw PJ, Ince PG.

Neuropathol Appl Neurobiol. 2014 Oct;40(6):670-85. doi: 10.1111/nan.12148.

PMID:
24750229
6.

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.

7.

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.

8.

Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death.

Xiao S, Sanelli T, Chiang H, Sun Y, Chakrabartty A, Keith J, Rogaeva E, Zinman L, Robertson J.

Acta Neuropathol. 2015 Jul;130(1):49-61. doi: 10.1007/s00401-015-1412-5. Epub 2015 Mar 19.

9.

Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.

Robertson J, Sanelli T, Xiao S, Yang W, Horne P, Hammond R, Pioro EP, Strong MJ.

Neurosci Lett. 2007 Jun 13;420(2):128-32. Epub 2007 Apr 8.

PMID:
17543992
10.

MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.

MacNair L, Xiao S, Miletic D, Ghani M, Julien JP, Keith J, Zinman L, Rogaeva E, Robertson J.

Brain. 2016 Jan;139(Pt 1):86-100. doi: 10.1093/brain/awv308. Epub 2015 Nov 2.

PMID:
26525917
11.

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.

12.

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
13.

Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations.

Finelli MJ, Liu KX, Wu Y, Oliver PL, Davies KE.

Hum Mol Genet. 2015 Jun 15;24(12):3529-44. doi: 10.1093/hmg/ddv104. Epub 2015 Mar 19.

14.

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.

15.

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity.

Johnson BS, Snead D, Lee JJ, McCaffery JM, Shorter J, Gitler AD.

J Biol Chem. 2009 Jul 24;284(30):20329-39. doi: 10.1074/jbc.M109.010264. Epub 2009 May 22. Erratum in: J Biol Chem. 2009 Sep 11;284(37):25459.

16.

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
17.

The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.

Collins M, Riascos D, Kovalik T, An J, Krupa K, Krupa K, Hood BL, Conrads TP, Renton AE, Traynor BJ, Bowser R.

Acta Neuropathol. 2012 Nov;124(5):717-32. doi: 10.1007/s00401-012-1045-x. Epub 2012 Sep 21.

18.

TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations.

Maekawa S, Leigh PN, King A, Jones E, Steele JC, Bodi I, Shaw CE, Hortobagyi T, Al-Sarraj S.

Neuropathology. 2009 Dec;29(6):672-83. doi: 10.1111/j.1440-1789.2009.01029.x. Epub 2009 Jun 3.

PMID:
19496940
19.

Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.

Wang W, Arakawa H, Wang L, Okolo O, Siedlak SL, Jiang Y, Gao J, Xie F, Petersen RB, Wang X.

Mol Ther. 2017 Jan 4;25(1):127-139. doi: 10.1016/j.ymthe.2016.10.013. Epub 2017 Jan 4.

PMID:
28129109
20.

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.

Turner BJ, Bäumer D, Parkinson NJ, Scaber J, Ansorge O, Talbot K.

BMC Neurosci. 2008 Oct 28;9:104. doi: 10.1186/1471-2202-9-104.

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