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How do C9ORF72 repeat expansions cause amyotrophic lateral sclerosis and frontotemporal dementia 由 Marka van Blitterswijk, Mariely DeJesus‐Hernandez, Rosa Rademakers

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Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65 由 Xin Sun, Pan P. Li, Shanshan Zhu, Rachael Cohen, Leonard O. Marque, Christopher A. Ross, Stefan M. Pulst, Ho Yin Edwin Chan, Russell L. Margolis, Dobrila D. Rudnicki

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Suppression of Somatic Expansion Delays the Onset of Pathophysiology in a Mouse Model of Huntington’s Disease 由 Helen Budworth, Faye R. Harris, Paul D. Williams, Do Yup Lee, Amy Holt, Jens Pahnke, Bartosz Szczesny, Karina Acevedo‐Torres, Sylvette Ayala‐Peña, Cynthia T. McMurray

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Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS 由 Ana Jovičić, Jérôme Mertens, Steven Boeynaems, Elke Bogaert, Noori Chai, Shizuka Yamada, Joseph W. Paul, Shuying Sun, Joseph R. Herdy, Gregor Bieri, Nicholas J. Kramer, Fred H. Gage, Ludo Van Den Bosch, Wim Robberecht, Aaron D. Gitler

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Identification and Characterization of Modified Antisense Oligonucleotides Targeting <i>DMPK</i> in Mice and Nonhuman Primates for the Treatment of Myotonic Dystrophy Type 1 由 Sanjay K. Pandey, Thurman M. Wheeler, Samantha L. Justice, Aneeza Kim, Husam S. Younis, Danielle Gattis, Dominic Jauvin, Jack Puymirat, Eric E. Swayze, Susan M. Freier, C. Frank Bennett, Charles A. Thornton, A. Robert MacLeod

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A Brief History of Triplet Repeat Diseases 由 Helen Budworth, Cynthia T. McMurray

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Cortical Function in Asymptomatic Carriers and Patients With<i>C9orf72</i>Amyotrophic Lateral Sclerosis 由 Nimeshan Geevasinga, Parvathi Menon, Garth A. Nicholson, Karl Ng, James Howells, Jillian J. Kril, Con Yiannikas, Matthew C. Kiernan, Steve Vucic

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GGA·TCC-interrupted Triplets in Long GAA·TTC Repeats Inhibit the Formation of Triplex and Sticky DNA Structures, Alleviate Transcription Inhibition, and Reduce Genetic Instabilitie... 由 Naoaki Sakamoto, Jacquelynn E. Larson, Ravi R. Iyer, Laura Montermini, Massimo Pandolfo, Robert D. Wells

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Induced Pluripotent Stem Cells from Patients with Huntington's Disease Show CAG-Repeat-Expansion-Associated Phenotypes 由 Virginia B. Mattis, Soshana Svendsen, Allison D. Ebert, Clive N. Svendsen, Alvin R. King, Malcolm Casale, Sara T. Winokur, Gayani Batugedara, Marquis P. Vawter, Peter J. Donovan, Leslie F. Lock, Leslie M. Thompson, Yu Zhu, Elisa Fossale, Ranjit Singh Atwal, Tammy Gillis, Jayalakshmi Srinidhi Mysore, Jianhong Li, Ihnsik Seong, Yiping Shen, Xiaoli Chen, Vanessa C. Wheeler, Marcy E. MacDonald, James F. Gusella, Sergey Akimov, Nicolas Arbez, Tarja Juopperi, Tamara Ratovitski, Jason Chiang, Woon Roung Kim, Eka Chighladze, Erin E. Watkin, Chun Zhong, Georgia Makri, Robert N. Cole, Russell L. Margolis, Hongjun Song, Guo‐li Ming, Christopher A. Ross, Julia Kaye, Aaron Daub, Punita Sharma, Amanda R. Mason, Steven Finkbeiner, Junying Yu, J. Allan Thomson, David J. Rushton, Stephen P. Brazier, Alysia Battersby, Amanda Redfern, Hsui Er Tseng, Alexander W. Harrison, Paul J. Kemp, Nicholas D. Allen, Marco Onorati, Valentina Castiglioni, Elena Cattaneo, Jamshid Arjomand

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Machado–Joseph disease/spinocerebellar ataxia type 3 由 Henry L. Paulson

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<i>C9ORF72</i> repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits 由 Jeannie Chew, Tania F. Gendron, Mercedes Prudencio, Hiroki Sasaguri, Yong‐Jie Zhang, Monica Castanedes‐Casey, Chris W. Lee, Karen Jansen‐West, Aishe Kurti, Melissa E. Murray, Kevin F. Bieniek, Peter Bauer, Ena C. Whitelaw, Linda Rousseau, Jeannette N. Stankowski, Caroline Stetler, Lillian M. Daughrity, Emilie A. Perkerson, Pamela Desaro, Amelia Johnston, Karen Overstreet, Dieter Edbauer, Rosa Rademakers, Khrista Boylan, Dennis W. Dickson, John Denis Fryer, Leonard Petrucelli

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Ubiquitin–proteasome system involvement in Huntington’s disease 由 Zaira Ortega, José J. Lucas

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Jump from Pre-mutation to Pathologic Expansion in C9orf72 由 Zhengrui Xi, Marka van Blitterswijk, Ming Zhang, Philip McGoldrick, Jesse R. McLean, Yana Yunusova, Erin Knock, Danielle Moreno, Christine Sato, Paul M. McKeever, Raphaël Schneider, Julia Keith, Nicolae Petrescu, Paul E. Fraser, Maria Carmela Tartaglia, Matthew Baker, Neill R. Graff‐Radford, Khrista Boylan, Dennis W. Dickson, Ian R. Mackenzie, Rosa Rademakers, Janice Robertson, Lorne Zinman, Ekaterina Rogaeva

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Personalized gene silencing therapeutics for Huntington disease 由 Chris Kay, Niels H. Skotte, Amber L. Southwell, Michael R. Hayden

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Neuropathology and <scp>C</scp>ellular <scp>P</scp>athogenesis of <scp>S</scp>pinocerebellar <scp>A</scp>taxia <scp>T</scp>ype 12 由 Elizabeth O’Hearn, Hyon S. Hwang, Susan E. Holmes, Dobrila D. Rudnicki, Daniel W. Chung, Ana I. Seixas, Rachael Cohen, Christopher A. Ross, John Q. Trojanowski, Olga Pletniková, Juan C. Troncoso, Russell L. Margolis

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Muscle wasting in myotonic dystrophies: a model of premature aging 由 Alba Judith Mateos-Aierdi, María Goicoechea, Ana Aiastui, Roberto Fernández‐Torrón, Mikel García-Puga, Ander Matheu, Adolfo López de Munaín

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CAG Repeat Expansion in the Androgen Receptor Gene Is Not Associated With Male Infertility in Indian Populations 由 Kumarasamy Thangaraj, Manjunath B. Joshi, Alla G. Reddy, Nalini Gupta, Baidyanath Chakravarty, Lalji Singh

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Huntington’s disease cerebrospinal fluid seeds aggregation of mutant huntingtin 由 Zhiqun Tan, Wei Dai, Theo G.M. van Erp, Julia Overman, Angelo Demuro, Michelle A. Digman, Asa Hatami, Ricardo Albay, Emily M. Sontag, Katya T Potkin, Shuo‐Chien Ling, Fabìo Macciardi, W.E. Bunney, Jeffrey D. Long, Jane S. Paulsen, John M. Ringman, Ian Parker, Charles Glabe, Leslie M. Thompson, Wah Chiu, Steven G. Potkin

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Larger Trinucleotide Repeat Size in the Androgen Receptor Gene of Infertile Men With Extremely Severe Oligozoospermia 由 Pasquale Patrizio, Debra G. B. Leonard, Ke‐Lian Chen, S. Hernández-Ayup, Alan Trounson

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Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load 由 Austen J. Milnerwood, Lynn A. Raymond

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