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Select item 50448291.

NF-κB and IRF1 Induce Endogenous Retrovirus K Expression via Interferon-Stimulated Response Elements in Its 5′ Long Terminal Repeat

Mamneet Manghera, Jennifer Ferguson-Parry, Rongtuan Lin, Renée N. Douville

J Virol. 2016 Oct 15; 90(20): 9338–9349. Prepublished online 2016 Aug 10. Published online 2016 Sep 29. doi: 10.1128/JVI.01503-16

PMCID:: PMC5044829

Abstract Article PDF–3.7M

Select item 48334352.

Cryptic Amyloidogenic Elements in the 3′ UTRs of Neurofilament Genes Trigger Axonal Neuropathy

Adriana P. Rebelo, Alexander J. Abrams, Ellen Cottenie, Alejandro Horga, Michael Gonzalez, Dana M. Bis, Avencia Sanchez-Mejias, Milena Pinto, Elena Buglo, Kasey Markel, Jeffrey Prince, Matilde Laura, Henry Houlden, Julian Blake, Cathy Woodward, Mary G. Sweeney, Janice L. Holton, Michael Hanna, Julia E. Dallman, Michaela Auer-Grumbach, Mary M. Reilly, Stephan Zuchner

Am J Hum Genet. 2016 Apr 7; 98(4): 597–614. Published online 2016 Mar 31. doi: 10.1016/j.ajhg.2016.02.022

PMCID:: PMC4833435

Abstract Article PDF–3.5M

Select item 48614593.

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 48626214.

Genetic analysis of the FUS/TLS gene in essential tremor

N. Parmalee, K. Mirzozoda, S. Kisselev, N. Merner, P. Dion, G. Rouleau, L. Clark, E. D. Louis

Eur J Neurol. Author manuscript; available in PMC 2016 May 10.

Published in final edited form as: Eur J Neurol. 2013 Mar; 20(3): 534–539. Published online 2012 Nov 1. doi: 10.1111/ene.12023

PMCID:: PMC4862621

Abstract Article PDF–314K

Select item 48659285.

Enhancing NAD⁺ Salvage Pathway Reverts the Toxicity of Primary Astrocytes Expressing Amyotrophic Lateral Sclerosis-linked Mutant Superoxide Dismutase 1 (SOD1)

Benjamin A. Harlan, Mariana Pehar, Deep R. Sharma, Gyda Beeson, Craig C. Beeson, Marcelo R. Vargas

J Biol Chem. 2016 May 13; 291(20): 10836–10846. Published online 2016 Mar 21. doi: 10.1074/jbc.M115.698779

PMCID:: PMC4865928

Abstract Article PDF–2.6M

Select item 48702726.

Bid Promotes K63-Linked Polyubiquitination of Tumor Necrosis Factor Receptor Associated Factor 6 (TRAF6) and Sensitizes to Mutant SOD1-Induced Proinflammatory Signaling in Microglia

Sinéad Kinsella, Hans-Georg König, Jochen H.M. Prehn

eNeuro. 2016 Mar-Apr; 3(2): ENEURO.0099-15.2016. Prepublished online 2016 May 2. Published online 2016 May 12. doi: 10.1523/ENEURO.0099-15.2016

PMCID:: PMC4870272

Abstract Article PDF–2.3M

Select item 49181027.

Progressive impairment of Ca_V1.1 function in the skeletal muscle of mice expressing a mutant type 1 Cu/Zn superoxide dismutase (G93A) linked to amyotrophic lateral sclerosis

Donald Beqollari, Christin F. Romberg, Gabriella Dobrowolny, Martina Martini, Andrew A. Voss, Antonio Musarò, Roger A. Bannister

Skelet Muscle. 2016; 6: 24. Published online 2016 Jun 23. doi: 10.1186/s13395-016-0094-6

PMCID:: PMC4918102

Abstract Article PDF–10M

Select item 49245708.

Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis

KL Jackson, RD Dayton, EA Orchard, S Ju, D Ringe, GA Petsko, LE Maquat, RL Klein

Gene Ther. Author manuscript; available in PMC 2016 Jun 28.

Published in final edited form as: Gene Ther. 2015 Jan; 22(1): 20–28. Published online 2014 Nov 6. doi: 10.1038/gt.2014.101

PMCID:: PMC4924570

Abstract Article PDF–578K

Select item 49424189.

Mutant SOD1 protein increases Na_v1.3 channel excitability

Elif Kubat Öktem, Karen Mruk, Joshua Chang, Ata Akin, William R. Kobertz, Robert H. Brown, Jr.

J Biol Phys. 2016 Jun; 42(3): 351–370. Published online 2016 Apr 12. doi: 10.1007/s10867-016-9411-x

Correction in:: J Biol Phys. 2016 Jun; 42(3): 371.

PMCID:: PMC4942418

Abstract Article PDF–1.7M

Select item 494501010.

Amyotrophic Lateral Sclerosis Type 20 - In Silico Analysis and Molecular Dynamics Simulation of hnRNPA1

Bruna Baumgarten Krebs, Joelma Freire De Mesquita

PLoS One. 2016; 11(7): e0158939. Published online 2016 Jul 14. doi: 10.1371/journal.pone.0158939

PMCID:: PMC4945010

Abstract Article PDF–5.6M

Select item 498633411.

Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation

Kohsuke Kanekura, Takuya Yagi, Alexander J. Cammack, Jana Mahadevan, Masahiko Kuroda, Matthew B. Harms, Timothy M. Miller, Fumihiko Urano

Hum Mol Genet. 2016 May 1; 25(9): 1803–1813. Published online 2016 Feb 29. doi: 10.1093/hmg/ddw052

PMCID:: PMC4986334

Abstract Article PDF–1.0M

Select item 501502312.

Screening of SOD1, FUS and TARDBP genes in patients with amyotrophic lateral sclerosis in central-southern China

Lihua Hou, Bin Jiao, Tingting Xiao, Lu Zhou, Zhifan Zhou, Juan Du, Xinxiang Yan, Junling Wang, Beisha Tang, Lu Shen

Sci Rep. 2016; 6: 32478. Published online 2016 Sep 8. doi: 10.1038/srep32478

PMCID:: PMC5015023

Abstract Article PDF–913K

Select item 501612113.

Cellular Redox Systems Impact the Aggregation of Cu,Zn Superoxide Dismutase Linked to Familial Amyotrophic Lateral Sclerosis

Cristina Álvarez-Zaldiernas, Jun Lu, Yujuan Zheng, Hongqian Yang, Juan Blasi, Carles Solsona, Arne Holmgren

J Biol Chem. 2016 Aug 12; 291(33): 17197–17208. Published online 2016 Jun 3. doi: 10.1074/jbc.M115.708230

PMCID:: PMC5016121

Abstract Article PDF–3.1M

Select item 478777314.

Quadruplex formation by both G-rich and C-rich DNA strands of the C9orf72 (GGGGCC)8•(GGCCCC)8 repeat: effect of CpG methylation

Bita Zamiri, Mila Mirceta, Karol Bomsztyk, Robert B. Macgregor, Jr., Christopher E. Pearson

Nucleic Acids Res. 2015 Nov 16; 43(20): 10055–10064. Published online 2015 Oct 1. doi: 10.1093/nar/gkv1008

PMCID:: PMC4787773

Abstract Article PDF–5.8M

Select item 505639615.

Hexokinase I N-terminal based peptide prevents the VDAC1-SOD1 G93A interaction and re-establishes ALS cell viability

Andrea Magrì, Ramona Belfiore, Simona Reina, Marianna Flora Tomasello, Maria Carmela Di Rosa, Francesca Guarino, Loredana Leggio, Vito De Pinto, Angela Messina

Sci Rep. 2016; 6: 34802. Published online 2016 Oct 10. doi: 10.1038/srep34802

PMCID:: PMC5056396

Abstract Article PDF–1.5M

Select item 523379416.

The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion

Emma M Devenney, Ramon Landin-Romero, Muireann Irish, Michael Hornberger, Eneida Mioshi, Glenda M. Halliday, Matthew C. Kiernan, John R. Hodges

Neuroimage Clin. 2017; 13: 439–445. Published online 2016 Dec 2. doi: 10.1016/j.nicl.2016.11.028

PMCID:: PMC5233794

Abstract Article PDF–871K

Select item 526447617.

TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis

Stephanie M. Williams, Galam Khan, Brent T. Harris, John Ravits, Michael R. Sierks

BMC Neurosci. 2017; 18: 20. Published online 2017 Jan 25. doi: 10.1186/s12868-017-0334-7

PMCID:: PMC5264476

Abstract Article PDF–1.6M

Select item 530221518.

ATXN2 trinucleotide repeat length correlates with risk of ALS

William Sproviero, Aleksey Shatunov, Daniel Stahl, Maryam Shoai, Wouter van Rheenen, Ashley R. Jones, Safa Al-Sarraj, Peter M. Andersen, Nancy M. Bonini, Francesca L. Conforti, Philip Van Damme, Hussein Daoud, Maria Del Mar Amador, Isabella Fogh, Monica Forzan, Ben Gaastra, Cinzia Gellera, Aaron D. Gitler, John Hardy, Pietro Fratta, Vincenzo La Bella, Isabelle Le Ber, Tim Van Langenhove, Serena Lattante, Yi-Chung Lee, Andrea Malaspina, Vincent Meininger, Stéphanie Millecamps, Richard Orrell, Rosa Rademakers, Wim Robberecht, Guy Rouleau, Owen A. Ross, Francois Salachas, Katie Sidle, Bradley N. Smith, Bing-Wen Soong, Gianni Sorarù, Giovanni Stevanin, Edor Kabashi, Claire Troakes, Christine van Broeckhoven, Jan H. Veldink, Leonard H. van den Berg, Christopher E. Shaw, John F. Powell, Ammar Al-Chalabi

Neurobiol Aging. 2017 Mar; 51: 178.e1–178.e9. doi: 10.1016/j.neurobiolaging.2016.11.010

PMCID:: PMC5302215

Abstract Article

Select item 534421119.

ApoSOD1 lacking dismutase activity neuroprotects motor neurons exposed to beta-methylamino-L-alanine through the Ca²⁺/Akt/ERK1/2 prosurvival pathway

Tiziana Petrozziello, Agnese Secondo, Valentina Tedeschi, Alba Esposito, MariaJosè Sisalli, Antonella Scorziello, Gianfranco Di Renzo, Lucio Annunziato

Cell Death Differ. 2017 Mar; 24(3): 511–522. Published online 2017 Jan 13. doi: 10.1038/cdd.2016.154

PMCID:: PMC5344211

Abstract Article PDF–3.4M

Select item 534575920.

The essential and downstream common proteins of amyotrophic lateral sclerosis: A protein-protein interaction network analysis

Yimin Mao, Su-Wei Kuo, Le Chen, C. J. Heckman, M. C. Jiang

PLoS One. 2017; 12(3): e0172246. Published online 2017 Mar 10. doi: 10.1371/journal.pone.0172246

PMCID:: PMC5345759

Abstract Article PDF–3.5M

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