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Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis.

Shang Y, Huang EJ.

Brain Res. 2016 Sep 15;1647:65-78. doi: 10.1016/j.brainres.2016.03.036. Epub 2016 Mar 28. Review.


Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains.

Kametani F, Obi T, Shishido T, Akatsu H, Murayama S, Saito Y, Yoshida M, Hasegawa M.

Sci Rep. 2016 Mar 16;6:23281. doi: 10.1038/srep23281.


Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils.

Shimonaka S, Nonaka T, Suzuki G, Hisanaga S, Hasegawa M.

J Biol Chem. 2016 Apr 22;291(17):8896-907. doi: 10.1074/jbc.M115.713552. Epub 2016 Feb 17.


Molecular Pathological Classification of Neurodegenerative Diseases: Turning towards Precision Medicine.

Kovacs GG.

Int J Mol Sci. 2016 Feb 2;17(2). pii: E189. doi: 10.3390/ijms17020189. Review.


Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43.

Nonaka T, Suzuki G, Tanaka Y, Kametani F, Hirai S, Okado H, Miyashita T, Saitoe M, Akiyama H, Masai H, Hasegawa M.

J Biol Chem. 2016 Mar 11;291(11):5473-83. doi: 10.1074/jbc.M115.695379. Epub 2016 Jan 14.


ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43.

Lim L, Wei Y, Lu Y, Song J.

PLoS Biol. 2016 Jan 6;14(1):e1002338. doi: 10.1371/journal.pbio.1002338. eCollection 2016 Jan.


Platform for high-throughput antibody selection using synthetically-designed antibody libraries.

Batonick M, Holland EG, Busygina V, Alderman D, Kay BK, Weiner MP, Kiss MM.

N Biotechnol. 2016 Sep 25;33(5 Pt A):565-73. doi: 10.1016/j.nbt.2015.11.005. Epub 2015 Nov 24.


Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin.

Amlie-Wolf A, Ryvkin P, Tong R, Dragomir I, Suh E, Xu Y, Van Deerlin VM, Gregory BD, Kwong LK, Trojanowski JQ, Lee VM, Wang LS, Lee EB.

PLoS One. 2015 Oct 28;10(10):e0141836. doi: 10.1371/journal.pone.0141836. eCollection 2015.


Modeling ALS and FTD with iPSC-derived neurons.

Lee S, Huang EJ.

Brain Res. 2015 Oct 14. pii: S0006-8993(15)00740-4. doi: 10.1016/j.brainres.2015.10.003. [Epub ahead of print] Review.


Fragile X protein mitigates TDP-43 toxicity by remodeling RNA granules and restoring translation.

Coyne AN, Yamada SB, Siddegowda BB, Estes PS, Zaepfel BL, Johannesmeyer JS, Lockwood DB, Pham LT, Hart MP, Cassel JA, Freibaum B, Boehringer AV, Taylor JP, Reitz AB, Gitler AD, Zarnescu DC.

Hum Mol Genet. 2015 Dec 15;24(24):6886-98. doi: 10.1093/hmg/ddv389. Epub 2015 Sep 18.


Activation of ER Stress and Autophagy Induced by TDP-43 A315T as Pathogenic Mechanism and the Corresponding Histological Changes in Skin as Potential Biomarker for ALS with the Mutation.

Wang X, Zhou S, Ding X, Ma M, Zhang J, Zhou Y, Wu E, Teng J.

Int J Biol Sci. 2015 Jul 21;11(10):1140-9. doi: 10.7150/ijbs.12657. eCollection 2015.


An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies.

Coan G, Mitchell CS.

Neurodegener Dis. 2015;15(5):301-12. doi: 10.1159/000433581. Epub 2015 Jul 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.


The function of RNA-binding proteins at the synapse: implications for neurodegeneration.

Sephton CF, Yu G.

Cell Mol Life Sci. 2015 Oct;72(19):3621-35. doi: 10.1007/s00018-015-1943-x. Epub 2015 Jun 6. Review.


A network of RNA and protein interactions in Fronto Temporal Dementia.

Fontana F, Siva K, Denti MA.

Front Mol Neurosci. 2015 Mar 19;8:9. doi: 10.3389/fnmol.2015.00009. eCollection 2015. Review.


TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.

Scotter EL, Chen HJ, Shaw CE.

Neurotherapeutics. 2015 Apr;12(2):352-63. doi: 10.1007/s13311-015-0338-x. Review. Erratum in: Neurotherapeutics. 2015 Apr;12(2):515-8.


TDP-43 regulates β-adducin (Add2) transcript stability.

Costessi L, Porro F, Iaconcig A, Muro AF.

RNA Biol. 2014;11(10):1280-90. doi: 10.1080/15476286.2014.996081.


R-loops highlight the nucleus in ALS.

Salvi JS, Mekhail K.

Nucleus. 2015;6(1):23-9. doi: 10.1080/19491034.2015.1004952. Review.


Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine.

Irwin DJ, Cairns NJ, Grossman M, McMillan CT, Lee EB, Van Deerlin VM, Lee VM, Trojanowski JQ.

Acta Neuropathol. 2015 Apr;129(4):469-91. doi: 10.1007/s00401-014-1380-1. Epub 2014 Dec 31.

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