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Select item 101754331.

C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD

Pedro Ervilha Pereira, Nika Schuermans, Antoon Meylemans, Pontus LeBlanc, Lauren Versluys, Katie E. Copley, Jack D. Rubien, Christopher Altheimer, Myra Peetermans, Elke Debackere, Olivier Vanakker, Sandra Janssens, Jonathan Baets, Kristof Verhoeven, Martin Lammens, Sofie Symoens, Boel De Paepe, Sami J. Barmada, James Shorter, Jan L. De Bleecker, Elke Bogaert, Bart Dermaut

Acta Neuropathol. 2023; 145(6): 793–814. Published online 2023 Mar 31. doi: 10.1007/s00401-023-02565-1

PMCID:: PMC10175433

Abstract Article PDF–6.1M

Select item 71388392.

Astrocytes with TDP-43 inclusions exhibit reduced noradrenergic cAMP and Ca²⁺ signaling and dysregulated cell metabolism

Jelena Velebit, Anemari Horvat, Tina Smolič, Sonja Prpar Mihevc, Boris Rogelj, Robert Zorec, Nina Vardjan

Sci Rep. 2020; 10: 6003. Published online 2020 Apr 7. doi: 10.1038/s41598-020-62864-5

PMCID:: PMC7138839

Abstract Article PDF–7.1M

Select item 71166503.

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

Jemeen Sreedharan, Ian P. Blair, Vineeta B. Tripathi, Xun Hu, Caroline Vance, Boris Rogelj, Steven Ackerley, Jennifer C. Durnall, Kelly L. Williams, Emanuele Buratti, Francisco Baralle, Jacqueline de Belleroche, J. Douglas Mitchell, P. Nigel Leigh, Ammar Al-Chalabi, Christopher C. Miller, Garth Nicholson, Christopher E. Shaw

Science. Author manuscript; available in PMC 2021 Jan 29.

Published in final edited form as: Science. 2008 Mar 21; 319(5870): 1668–72. Published online 2008 Feb 28. doi: 10.1126/science.1154584

PMCID:: PMC7116650

Abstract Article PDF–1.3M

Select item 68348924.

TDP-43 knockdown causes innate immune activation via protein kinase R in astrocytes

Thomas J. LaRocca, Andrea Mariani, Linda R. Watkins, Christopher D. Link

Neurobiol Dis. Author manuscript; available in PMC 2020 Dec 1.

Published in final edited form as: Neurobiol Dis. 2019 Dec; 132: 104514. Published online 2019 Jun 21. doi: 10.1016/j.nbd.2019.104514

PMCID:: PMC6834892

Abstract Article PDF–1.2M

Select item 60560155.

Atomic-level evidence for packing and positional amyloid polymorphism by segment from TDP-43 RRM2

Elizabeth L. Guenther, Peng Ge, Hamilton Trinh, Michael R. Sawaya, Duilio Cascio, David R. Boyer, Tamir Gonen, Z. Hong Zhou, David S. Eisenberg

Nat Struct Mol Biol. Author manuscript; available in PMC 2018 Oct 1.

Published in final edited form as: Nat Struct Mol Biol. 2018 Apr; 25(4): 311–319. Published online 2018 Mar 12. doi: 10.1038/s41594-018-0045-5

PMCID:: PMC6056015

Abstract Article PDF–1.2M

Select item 59978436.

Temporal Expression of Mutant TDP-43 Correlates with Early Amyotrophic Lateral Sclerosis Phenotype and Motor Weakness

Qihua Chen, Jinxia Zhou, Cao Huang, Bo Huang, Fangfang Bi, Hongxia Zhou, Bo Xiao

Curr Neurovasc Res. 2018 Feb; 15(1): 3–9. Published online 2018 Feb. doi: 10.2174/1567202615666180109161541

PMCID:: PMC5997843

Abstract Article PDF–2.6M

Select item 58546327.

TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia

Hilary C. Archbold, Kasey L. Jackson, Ayush Arora, Kaitlin Weskamp, Elizabeth M.-H. Tank, Xingli Li, Roberto Miguez, Robert D. Dayton, Sharon Tamir, Ronald L. Klein, Sami J. Barmada

Sci Rep. 2018; 8: 4606. Published online 2018 Mar 15. doi: 10.1038/s41598-018-22858-w

PMCID:: PMC5854632

Abstract Article PDF–6.4M

Select item 54671708.

Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrity

Jung-A. A. Woo, Tian Liu, Courtney Trotter, Cenxiao C. Fang, Emillio De Narvaez, Patrick LePochat, Drew Maslar, Anusha Bukhari, Xingyu Zhao, Andrew Deonarine, Sandy D. Westerheide, David E. Kang

Nat Commun. 2017; 8: 15558. Published online 2017 Jun 6. doi: 10.1038/ncomms15558

PMCID:: PMC5467170

Abstract Article PDF–3.0M

Select item 54495909.

TDP-43 suppresses tau expression via promoting its mRNA instability

Jianlan Gu, Feng Wu, Wen Xu, Jianhua Shi, Wen Hu, Nana Jin, Wei Qian, Xinglong Wang, Khalid Iqbal, Cheng-Xin Gong, Fei Liu

Nucleic Acids Res. 2017 Jun 2; 45(10): 6177–6193. Published online 2017 Mar 15. doi: 10.1093/nar/gkx175

PMCID:: PMC5449590

Abstract Article PDF–8.8M

Select item 537340810.

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

Pallavi P. Gopal, Jeffrey J. Nirschl, Eva Klinman, Erika L. F. Holzbaur

Proc Natl Acad Sci U S A. 2017 Mar 21; 114(12): E2466–E2475. Published online 2017 Mar 6. doi: 10.1073/pnas.1614462114

PMCID:: PMC5373408

Abstract Article PDF–2.2M

Select item 493597411.

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses

Katannya Kapeli, Gabriel A. Pratt, Anthony Q. Vu, Kasey R. Hutt, Fernando J. Martinez, Balaji Sundararaman, Ranjan Batra, Peter Freese, Nicole J. Lambert, Stephanie C. Huelga, Seung J. Chun, Tiffany Y. Liang, Jeremy Chang, John P. Donohue, Lily Shiue, Jiayu Zhang, Haining Zhu, Franca Cambi, Edward Kasarskis, Shawn Hoon, Manuel Ares Jr., Christopher B. Burge, John Ravits, Frank Rigo, Gene W. Yeo

Nat Commun. 2016; 7: 12143. Published online 2016 Jul 5. doi: 10.1038/ncomms12143

PMCID:: PMC4935974

Abstract Article PDF–1.7M

Select item 486145912.

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

Shotaro Shimonaka, Takashi Nonaka, Genjiro Suzuki, Shin-ichi Hisanaga, Masato Hasegawa

J Biol Chem. 2016 Apr 22; 291(17): 8896–8907. Published online 2016 Feb 17. doi: 10.1074/jbc.M115.713552

PMCID:: PMC4861459

Abstract Article PDF–3.1M

Select item 484977413.

USP7 and TDP-43: Pleiotropic Regulation of Cryptochrome Protein Stability Paces the Oscillation of the Mammalian Circadian Clock

Arisa Hirano, Tomoki Nakagawa, Hikari Yoshitane, Masaaki Oyama, Hiroko Kozuka-Hata, Darin Lanjakornsiripan, Yoshitaka Fukada

PLoS One. 2016; 11(4): e0154263. Published online 2016 Apr 28. doi: 10.1371/journal.pone.0154263

PMCID:: PMC4849774

Abstract Article PDF–2.2M

Select item 470330714.

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

Liangzhong Lim, Yuanyuan Wei, Yimei Lu, Jianxing Song

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

PMCID:: PMC4703307

Abstract Article PDF–3.7M

Select item 415866115.

Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury

Zhihui Yang, Fan Lin, Claudia S Robertson, Kevin K W Wang

J Cereb Blood Flow Metab. 2014 Sep; 34(9): 1444–1452. Published online 2014 Jun 11. doi: 10.1038/jcbfm.2014.105

PMCID:: PMC4158661

Abstract Article PDF–641K

Select item 415568916.

Phosphorylation and Degradation of Tomosyn-2 De-represses Insulin Secretion

Sushant Bhatnagar, Mufaddal S. Soni, Lindsay S. Wrighton, Alexander S. Hebert, Amber S. Zhou, Pradyut K. Paul, Trillian Gregg, Mary E. Rabaglia, Mark P. Keller, Joshua J. Coon, Alan D. Attie

J Biol Chem. 2014 Sep 5; 289(36): 25276–25286. Published online 2014 Jul 7. doi: 10.1074/jbc.M114.575985

PMCID:: PMC4155689

Abstract Article PDF–1.1M

Select item 406637217.

Profiling the Genes Affected by Pathogenic TDP-43 in Astrocytes

Cao Huang, Bo Huang, Fangfang Bi, Linda H Yan, Jianbin Tong, Jufang Huang, Xu-Gang Xia, Hongxia Zhou

J Neurochem. Author manuscript; available in PMC 2015 Jun 1.

Published in final edited form as: J Neurochem. 2014 Jun; 129(6): 932–939. Published online 2014 Feb 9. doi: 10.1111/jnc.12660

PMCID:: PMC4066372

Abstract Article PDF–1.7M

Select item 398118118.

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

Jianbin Tong, Cao Huang, Fangfang Bi, Qinxue Wu, Bo Huang, Xionghao Liu, Fang Li, Hongxia Zhou, Xu-Gang Xia

EMBO J. 2013 Jul 3; 32(13): 1917–1926. Published online 2013 May 28. doi: 10.1038/emboj.2013.122

PMCID:: PMC3981181

Abstract Article

Select item 397933319.

Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans

Victoria E. Johnson, William Stewart, John Q. Trojanowski, Douglas H. Smith

Acta Neuropathol. Author manuscript; available in PMC 2014 Apr 8.

Published in final edited form as: Acta Neuropathol. 2011 Dec; 122(6): 715–726. Published online 2011 Nov 19. doi: 10.1007/s00401-011-0909-9

PMCID:: PMC3979333

Abstract Article PDF–3.4M

Select item 396046320.

ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor

LiQun Liu-Yesucevitz, Amy Y. Lin, Atsushi Ebata, Joon Y. Boon, Whitney Reid, Ya-Fei Xu, Kendra Kobrin, George J. Murphy, Leonard Petrucelli, Benjamin Wolozin

J Neurosci. 2014 Mar 19; 34(12): 4167–4174. doi: 10.1523/JNEUROSCI.2350-13.2014

PMCID:: PMC3960463

Abstract Article PDF–1.2M

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