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Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72
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.
Published in Nature neuroscience (01.05.2020)
Published in Nature neuroscience (01.05.2020)
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Journal Article
Spinal subpial delivery of AAV9 enables widespread gene silencing and blocks motoneuron degeneration in ALS
Bravo-Hernandez, Mariana, Tadokoro, Takahiro, Navarro, Michael R., Platoshyn, Oleksandr, Kobayashi, Yoshiomi, Marsala, Silvia, Miyanohara, Atsushi, Juhas, Stefan, Juhasova, Jana, Skalnikova, Helena, Tomori, Zoltan, Vanicky, Ivo, Studenovska, Hana, Proks, Vladimir, Chen, PeiXi, Govea-Perez, Noe, Ditsworth, Dara, Ciacci, Joseph D., Gao, Shang, Zhu, Wenlian, Ahrens, Eric T., Driscoll, Shawn P., Glenn, Thomas D., McAlonis-Downes, Melissa, Da Cruz, Sandrine, Pfaff, Samuel L., Kaspar, Brian K., Cleveland, Don W., Marsala, Martin
Published in Nature medicine (01.01.2020)
Published in Nature medicine (01.01.2020)
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Journal Article
Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis
Ling, Shuo-Chien, Dastidar, Somasish Ghosh, Tokunaga, Seiya, Ho, Wan Yun, Lim, Kenneth, Ilieva, Hristelina, Parone, Philippe A, Tyan, Sheue-Houy, Tse, Tsemay M, Chang, Jer-Cherng, Platoshyn, Oleksandr, Bui, Ngoc B, Bui, Anh, Vetto, Anne, Sun, Shuying, McAlonis-Downes, Melissa, Han, Joo Seok, Swing, Debbie, Kapeli, Katannya, Yeo, Gene W, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E, Tucker-Kellogg, Greg, La Spada, Albert R, Lagier-Tourenne, Clotilde, Da Cruz, Sandrine, Cleveland, Don W
Published in eLife (12.02.2019)
Published in eLife (12.02.2019)
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Journal Article
Progressive spinal axonal degeneration and slowness in ALS2-deficient mice
Yamanaka, Koji, Miller, Timothy M., McAlonis-Downes, Melissa, Chun, Seung Joo, Cleveland, Don W.
Published in Annals of neurology (01.07.2006)
Published in Annals of neurology (01.07.2006)
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Journal Article
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs
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
Published in Neuron (Cambridge, Mass.) (04.05.2016)
Published in Neuron (Cambridge, Mass.) (04.05.2016)
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Journal Article
Therapeutically viable generation of neurons with antisense oligonucleotide suppression of PTB
Maimon, Roy, Chillon-Marinas, Carlos, Snethlage, Cedric E., Singhal, Sarthak M., McAlonis-Downes, Melissa, Ling, Karen, Rigo, Frank, Bennett, C. Frank, Da Cruz, Sandrine, Hnasko, Thomas S., Muotri, Alysson R., Cleveland, Don W.
Published in Nature neuroscience (01.08.2021)
Published in Nature neuroscience (01.08.2021)
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Journal Article
Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS
Sun, Shuying, Sun, Ying, Ling, Shuo-Chien, Ferraiuolo, Laura, McAlonis-Downes, Melissa, Zou, Yiyang, Drenner, Kevin, Wang, Yin, Ditsworth, Dara, Tokunaga, Seiya, Kopelevich, Alex, Kaspar, Brian K., Lagier-Tourenne, Clotilde, Cleveland, Don W.
Published in Proceedings of the National Academy of Sciences - PNAS (15.12.2015)
Published in Proceedings of the National Academy of Sciences - PNAS (15.12.2015)
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Journal Article
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration
Lagier-Tourenne, Clotilde, Baughn, Michael, Rigo, Frank, Sun, Shuying, Liu, Patrick, Li, Hai-Ri, Jiang, Jie, Watt, Andrew T., Chun, Seung, Katz, Melanie, Qiu, Jinsong, Sun, Ying, Ling, Shuo-Chien, Zhu, Qiang, Polymenidou, Magdalini, Drenner, Kevin, Artates, Jonathan W., McAlonis-Downes, Melissa, Markmiller, Sebastian, Hutt, Kasey R., Pizzo, Donald P., Cady, Janet, Harms, Matthew B., Baloh, Robert H., Vandenberg, Scott R., Yeo, Gene W., Fu, Xiang-Dong, Bennett, C. Frank, Cleveland, Don W., Ravits, John
Published in Proceedings of the National Academy of Sciences - PNAS (19.11.2013)
Published in Proceedings of the National Academy of Sciences - PNAS (19.11.2013)
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Journal Article
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43
Arnold, Eveline S., Ling, Shuo-Chien, Huelga, Stephanie C., Lagier-Tourenne, Clotilde, Polymenidou, Magdalini, Ditsworth, Dara, Kordasiewicz, Holly B., McAlonis-Downes, Melissa, Platoshyn, Oleksandr, Parone, Philippe A., Da Cruz, Sandrine, Clutario, Kevin M., Swing, Debbie, Tessarollo, Lino, Marsala, Martin, Shaw, Christopher E., Yeo, Gene W., Cleveland, Don W.
Published in Proceedings of the National Academy of Sciences - PNAS (19.02.2013)
Published in Proceedings of the National Academy of Sciences - PNAS (19.02.2013)
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Journal Article
Enhancing Mitochondrial Calcium Buffering Capacity Reduces Aggregation of Misfolded SOD1 and Motor Neuron Cell Death without Extending Survival in Mouse Models of Inherited Amyotrophic Lateral Sclerosis
Parone, Philippe A., Da Cruz, Sandrine, Han, Joo Seok, McAlonis-Downes, Melissa, Vetto, Anne P., Lee, Sandra K., Tseng, Eva, Cleveland, Don W.
Published in The Journal of neuroscience (13.03.2013)
Published in The Journal of neuroscience (13.03.2013)
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Journal Article
Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1
Israelson, Adrian, Ditsworth, Dara, Sun, Shuying, Song, SungWon, Liang, Jason, Hruska-Plochan, Marian, McAlonis-Downes, Melissa, Abu-Hamad, Salah, Zoltsman, Guy, Shani, Tom, Maldonado, Marcus, Bui, Anh, Navarro, Michael, Zhou, Huilin, Marsala, Martin, Kaspar, Brian K., Da Cruz, Sandrine, Cleveland, Don W.
Published in Neuron (Cambridge, Mass.) (08.04.2015)
Published in Neuron (Cambridge, Mass.) (08.04.2015)
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Journal Article
Frontotemporal dementia-like disease progression elicited by seeded aggregation and spread of FUS
Vazquez-Sanchez, Sonia, Tilkin, Britt, Gasset-Rosa, Fatima, Zhang, Sitao, Piol, Diana, McAlonis-Downes, Melissa, Artates, Jonathan, Govea-Perez, Noe, Verresen, Yana, Guo, Lin, Cleveland, Don W., Shorter, James, Da Cruz, Sandrine
Published in Molecular neurodegeneration (11.06.2024)
Published in Molecular neurodegeneration (11.06.2024)
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Journal Article
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice
Yamanaka, Koji, Boillee, Severine, Roberts, Elizabeth A, Garcia, Michael L, McAlonis-Downes, Melissa, Mikse, Oliver R, Cleveland, Don W, Goldstein, Lawrence S.B
Published in Proceedings of the National Academy of Sciences - PNAS (27.05.2008)
Published in Proceedings of the National Academy of Sciences - PNAS (27.05.2008)
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Journal Article
C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice
Lobsiger, Christian S., Boillée, Severine, Pozniak, Christine, Khan, Amir M., McAlonis-Downes, Melissa, Lewcock, Joseph W., Cleveland, Don W.
Published in Proceedings of the National Academy of Sciences - PNAS (12.11.2013)
Published in Proceedings of the National Academy of Sciences - PNAS (12.11.2013)
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Journal Article
Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset
Vande Velde, Christine, McDonald, Karli K., Boukhedimi, Yasmin, McAlonis-Downes, Melissa, Lobsiger, Christian S., Bel Hadj, Samar, Zandona, Andre, Julien, Jean-Pierre, Shah, Sameer B., Cleveland, Don W.
Published in PloS one (11.07.2011)
Published in PloS one (11.07.2011)
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Journal Article
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice
Lobsiger, Christian S, Boillee, Severine, McAlonis-Downes, Melissa, Khan, Amir M, Feltri, M. Laura, Yamanaka, Koji, Cleveland, Don W
Published in Proceedings of the National Academy of Sciences - PNAS (17.03.2009)
Published in Proceedings of the National Academy of Sciences - PNAS (17.03.2009)
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Journal Article
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation
Sanjuan-Ruiz, Inmaculada, Govea-Perez, Noé, McAlonis-Downes, Melissa, Dieterle, Stéphane, Megat, Salim, Dirrig-Grosch, Sylvie, Picchiarelli, Gina, Piol, Diana, Zhu, Qiang, Myers, Brian, Lee, Chao-Zong, Cleveland, Don W, Lagier-Tourenne, Clotilde, Da Cruz, Sandrine, Dupuis, Luc
Published in Molecular neurodegeneration (06.09.2021)
Published in Molecular neurodegeneration (06.09.2021)
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Journal Article
An endogenous peptide marker differentiates SOD1 stability and facilitates pharmacodynamic monitoring in SOD1 amyotrophic lateral sclerosis
Gertsman, Ilya, Wuu, Joanne, McAlonis-Downes, Melissa, Ghassemian, Majid, Ling, Karen, Rigo, Frank, Bennett, Frank, Benatar, Michael, Miller, Timothy M., Da Cruz, Sandrine
Published in JCI insight (16.05.2019)
Published in JCI insight (16.05.2019)
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Journal Article
ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS
López-Erauskin, Jone, Tadokoro, Takahiro, Baughn, Michael W., Myers, Brian, McAlonis-Downes, Melissa, Chillon-Marinas, Carlos, Asiaban, Joshua N., Artates, Jonathan, Bui, Anh T., Vetto, Anne P., Lee, Sandra K., Le, Ai Vy, Sun, Ying, Jambeau, Mélanie, Boubaker, Jihane, Swing, Deborah, Qiu, Jinsong, Hicks, Geoffrey G., Ouyang, Zhengyu, Fu, Xiang-Dong, Tessarollo, Lino, Ling, Shuo-Chien, Parone, Philippe A., Shaw, Christopher E., Marsala, Martin, Lagier-Tourenne, Clotilde, Cleveland, Don W., Da Cruz, Sandrine
Published in Neuron (Cambridge, Mass.) (21.11.2018)
Published in Neuron (Cambridge, Mass.) (21.11.2018)
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Journal Article
Misfolded SOD1 is not a primary component of sporadic ALS
Da Cruz, Sandrine, Bui, Anh, Saberi, Shahram, Lee, Sandra K., Stauffer, Jennifer, McAlonis-Downes, Melissa, Schulte, Derek, Pizzo, Donald P., Parone, Philippe A., Cleveland, Don W., Ravits, John
Published in Acta neuropathologica (01.07.2017)
Published in Acta neuropathologica (01.07.2017)
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Journal Article