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Items: 1 to 20 of 245

1.

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.

2.

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.

PMID:
21752789
3.

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.

4.

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS.

Al-Sarraj S, King A, Troakes C, Smith B, Maekawa S, Bodi I, Rogelj B, Al-Chalabi A, Hortobágyi T, Shaw CE.

Acta Neuropathol. 2011 Dec;122(6):691-702. doi: 10.1007/s00401-011-0911-2. Epub 2011 Nov 19.

PMID:
22101323
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.

6.

An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice.

Walker AK, Tripathy K, Restrepo CR, Ge G, Xu Y, Kwong LK, Trojanowski JQ, Lee VM.

Hum Mol Genet. 2015 Dec 20;24(25):7241-54. doi: 10.1093/hmg/ddv424. Epub 2015 Oct 16.

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.

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
9.

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.

10.

Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions.

Nishihira Y, Tan CF, Onodera O, Toyoshima Y, Yamada M, Morita T, Nishizawa M, Kakita A, Takahashi H.

Acta Neuropathol. 2008 Aug;116(2):169-82. doi: 10.1007/s00401-008-0385-z. Epub 2008 May 15.

PMID:
18481073
11.

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.

12.

Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.

Neumann M, Igaz LM, Kwong LK, Nakashima-Yasuda H, Kolb SJ, Dreyfuss G, Kretzschmar HA, Trojanowski JQ, Lee VM.

Acta Neuropathol. 2007 May;113(5):543-8. Epub 2007 Apr 6.

PMID:
17415574
13.

Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration.

Seelaar H, Klijnsma KY, de Koning I, van der Lugt A, Chiu WZ, Azmani A, Rozemuller AJ, van Swieten JC.

J Neurol. 2010 May;257(5):747-53. doi: 10.1007/s00415-009-5404-z. Epub 2009 Nov 28.

14.

Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy.

Thorpe JR, Tang H, Atherton J, Cairns NJ.

J Neural Transm (Vienna). 2008 Dec;115(12):1661-71. doi: 10.1007/s00702-008-0137-1. Epub 2008 Oct 31.

15.

Elevated TMEM106B levels exaggerate lipofuscin accumulation and lysosomal dysfunction in aged mice with progranulin deficiency.

Zhou X, Sun L, Brady OA, Murphy KA, Hu F.

Acta Neuropathol Commun. 2017 Jan 26;5(1):9. doi: 10.1186/s40478-017-0412-1.

16.

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.

17.

Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.

Kosaka T, Fu YJ, Shiga A, Ishidaira H, Tan CF, Tani T, Koike R, Onodera O, Nishizawa M, Kakita A, Takahashi H.

Neuropathology. 2012 Aug;32(4):373-84. doi: 10.1111/j.1440-1789.2011.01271.x. Epub 2011 Nov 21.

PMID:
22098653
18.

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies.

Geser F, Lee VM, Trojanowski JQ.

Neuropathology. 2010 Apr;30(2):103-12. doi: 10.1111/j.1440-1789.2009.01091.x. Epub 2010 Jan 25. Review.

19.

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
20.

TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.

Baloh RH.

FEBS J. 2011 Oct;278(19):3539-49. doi: 10.1111/j.1742-4658.2011.08256.x. Epub 2011 Aug 24. Review.

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