Amyotrophic Lateral Sclerosis: An Update for 2018 von Oskarsson, Björn, Gendron, Tania F., Staff, Nathan P.

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Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons von Lopez-Gonzalez, Rodrigo, Lu, Yubing, Gendron, Tania F., Karydas, Anna, Tran, Helene, Yang, Dejun, Petrucelli, Leonard, Miller, Bruce L., Almeida, Sandra, Gao, Fen-Biao

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Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS von Ash, Peter E.A., Bieniek, Kevin F., Gendron, Tania F., Caulfield, Thomas, Lin, Wen-Lang, DeJesus-Hernandez, Mariely, van Blitterswijk, Marka M., Jansen-West, Karen, Paul, Joseph W., Rademakers, Rosa, Boylan, Kevin B., Dickson, Dennis W., Petrucelli, Leonard

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Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs von Jiang, Jie, Zhu, Qiang, Gendron, Tania F., Saberi, Shahram, McAlonis-Downes, Melissa, Seelman, Amanda, Stauffer, Jennifer E., Jafar-nejad, Paymaan, Drenner, Kevin, Schulte, Derek, Chun, Seung, Sun, Shuying, Ling, Shuo-Chien, Myers, Brian, Engelhardt, Jeffery, Katz, Melanie, Baughn, Michael, Platoshyn, Oleksandr, Marsala, Martin, Watt, Andy, Heyser, Charles J., Ard, M. Colin, De Muynck, Louis, Daughrity, Lillian M., Swing, Deborah A., Tessarollo, Lino, Jung, Chris J., Delpoux, Arnaud, Utzschneider, Daniel T., Hedrick, Stephen M., de Jong, Pieter J., Edbauer, Dieter, Van Damme, Philip, Petrucelli, Leonard, Shaw, Christopher E., Bennett, C. Frank, Da Cruz, Sandrine, Ravits, John, Rigo, Frank, Cleveland, Don W., Lagier-Tourenne, Clotilde

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Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS von Su, Zhaoming, Zhang, Yongjie, Gendron, Tania F., Bauer, Peter O., Chew, Jeannie, Yang, Wang-Yong, Fostvedt, Erik, Jansen-West, Karen, Belzil, Veronique V., Desaro, Pamela, Johnston, Amelia, Overstreet, Karen, Oh, Seok-Yoon, Todd, Peter K., Berry, James D., Cudkowicz, Merit E., Boeve, Bradley F., Dickson, Dennis, Floeter, Mary Kay, Traynor, Bryan J., Morelli, Claudia, Ratti, Antonia, Silani, Vincenzo, Rademakers, Rosa, Brown, Robert H., Rothstein, Jeffrey D., Boylan, Kevin B., Petrucelli, Leonard, Disney, Matthew D.

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Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS von Tran, Helene, Almeida, Sandra, Moore, Jill, Gendron, Tania F., Chalasani, UmaDevi, Lu, Yubing, Du, Xing, Nickerson, Jeffrey A., Petrucelli, Leonard, Weng, Zhiping, Gao, Fen-Biao

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The role of tau in neurodegeneration von Gendron, Tania F, Petrucelli, Leonard

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD von O’Rourke, Jacqueline G., Bogdanik, Laurent, Muhammad, A.K.M.G., Gendron, Tania F., Kim, Kevin J., Austin, Andrew, Cady, Janet, Liu, Elaine Y., Zarrow, Jonah, Grant, Sharday, Ho, Ritchie, Bell, Shaughn, Carmona, Sharon, Simpkinson, Megan, Lall, Deepti, Wu, Kathryn, Daughrity, Lillian, Dickson, Dennis W., Harms, Matthew B., Petrucelli, Leonard, Lee, Edward B., Lutz, Cathleen M., Baloh, Robert H.

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Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72 von Zhu, Qiang, Jiang, Jie, Gendron, Tania F., McAlonis-Downes, Melissa, Jiang, Lulin, Taylor, Amy, Diaz Garcia, Sandra, Ghosh Dastidar, Somasish, Rodriguez, Maria J., King, Patrick, Zhang, Yongjie, La Spada, Albert R., Xu, Huaxi, Petrucelli, Leonard, Ravits, John, Da Cruz, Sandrine, Lagier-Tourenne, Clotilde, Cleveland, Don W.

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Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia von Prudencio, Mercedes, Humphrey, Jack, Pickles, Sarah, Brown, Anna-Leigh, Hill, Sarah E, Kachergus, Jennifer M, Shi, J, Heckman, Michael G, Spiegel, Matthew R, Cook, Casey, Song, Yuping, Yue, Mei, Daughrity, Lillian M, Carlomagno, Yari, Jansen-West, Karen, de Castro, Cristhoper Fernandez, DeTure, Michael, Koga, Shunsuke, Wang, Ying-Chih, Sivakumar, Prasanth, Bodo, Cristian, Candalija, Ana, Talbot, Kevin, Selvaraj, Bhuvaneish T, Burr, Karen, Chandran, Siddharthan, Newcombe, Jia, Lashley, Tammaryn, Hubbard, Isabel, Catalano, Demetra, Kim, Duyang, Propp, Nadia, Fennessey, Samantha, Fagegaltier, Delphine, Phatnani, Hemali, Secrier, Maria, Fisher, Elizabeth Mc, Oskarsson, Björn, van Blitterswijk, Marka, Rademakers, Rosa, Graff-Radford, Neil R, Boeve, Bradley F, Knopman, David S, Petersen, Ronald C, Josephs, Keith A, Thompson, E Aubrey, Raj, Towfique, Ward, Michael, Dickson, Dennis W, Gendron, Tania F, Fratta, Pietro, Petrucelli, Leonard

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Acetylation of the KXGS motifs in tau is a critical determinant in modulation of tau aggregation and clearance von Cook, Casey, Carlomagno, Yari, Gendron, Tania F, Dunmore, Judy, Scheffel, Kristyn, Stetler, Caroline, Davis, Mary, Dickson, Dennis, Jarpe, Matthew, DeTure, Michael, Petrucelli, Leonard

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Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD von Yuva-Aydemir, Yeliz, Almeida, Sandra, Krishnan, Gopinath, Gendron, Tania F., Gao, Fen-Biao

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Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis von Zhang, Yong-Jie, Gendron, Tania F., Ebbert, Mark T. W., O’Raw, Aliesha D., Yue, Mei, Jansen-West, Karen, Zhang, Xu, Prudencio, Mercedes, Chew, Jeannie, Cook, Casey N., Daughrity, Lillian M., Tong, Jimei, Song, Yuping, Pickles, Sarah R., Castanedes-Casey, Monica, Kurti, Aishe, Rademakers, Rosa, Oskarsson, Bjorn, Dickson, Dennis W., Hu, Wenqian, Gitler, Aaron D., Fryer, John D., Petrucelli, Leonard

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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS von Gendron, Tania F., Bieniek, Kevin F., Zhang, Yong-Jie, Jansen-West, Karen, Ash, Peter E. A., Caulfield, Thomas, Daughrity, Lillian, Dunmore, Judith H., Castanedes-Casey, Monica, Chew, Jeannie, Cosio, Danielle M., van Blitterswijk, Marka, Lee, Wing C., Rademakers, Rosa, Boylan, Kevin B., Dickson, Dennis W., Petrucelli, Leonard

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Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS von Prudencio, Mercedes, Belzil, Veronique V, Batra, Ranjan, Ross, Christian A, Gendron, Tania F, Pregent, Luc J, Murray, Melissa E, Overstreet, Karen K, Piazza-Johnston, Amelia E, Desaro, Pamela, Bieniek, Kevin F, DeTure, Michael, Lee, Wing C, Biendarra, Sherri M, Davis, Mary D, Baker, Matthew C, Perkerson, Ralph B, van Blitterswijk, Marka, Stetler, Caroline T, Rademakers, Rosa, Link, Christopher D, Dickson, Dennis W, Boylan, Kevin B, Li, Hu, Petrucelli, Leonard

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Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice von Peters, Owen M., Cabrera, Gabriela Toro, Tran, Helene, Gendron, Tania F., McKeon, Jeanne E., Metterville, Jake, Weiss, Alexandra, Wightman, Nicholas, Salameh, Johnny, Kim, Juhyun, Sun, Huaming, Boylan, Kevin B., Dickson, Dennis, Kennedy, Zachary, Lin, Ziqiang, Zhang, Yong-Jie, Daughrity, Lillian, Jung, Chris, Gao, Fen-Biao, Sapp, Peter C., Horvitz, H. Robert, Bosco, Daryl A., Brown, Solange P., de Jong, Pieter, Petrucelli, Leonard, Mueller, Christian, Brown, Robert H.

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Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers von Mackenzie, Ian R. A., Frick, Petra, Grässer, Friedrich A., Gendron, Tania F., Petrucelli, Leonard, Cashman, Neil R., Edbauer, Dieter, Kremmer, Elisabeth, Prudlo, Johannes, Troost, Dirk, Neumann, Manuela

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Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress von Zhang, Yong-Jie, Jansen-West, Karen, Xu, Ya-Fei, Gendron, Tania F., Bieniek, Kevin F., Lin, Wen-Lang, Sasaguri, Hiroki, Caulfield, Thomas, Hubbard, Jaime, Daughrity, Lillian, Chew, Jeannie, Belzil, Veronique V., Prudencio, Mercedes, Stankowski, Jeannette N., Castanedes-Casey, Monica, Whitelaw, Ena, Ash, Peter E. A., DeTure, Michael, Rademakers, Rosa, Boylan, Kevin B., Dickson, Dennis W., Petrucelli, Leonard

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Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood von Belzil, Veronique V., Bauer, Peter O., Prudencio, Mercedes, Gendron, Tania F., Stetler, Caroline T., Yan, Irene K., Pregent, Luc, Daughrity, Lillian, Baker, Matthew C., Rademakers, Rosa, Boylan, Kevin, Patel, Tushar C., Dickson, Dennis W., Petrucelli, Leonard

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Biomarkers for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Associated With Hexanucleotide Expansion Mutations in C9orf72 von Floeter, Mary Kay, Gendron, Tania F

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