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Times Cited: 2342
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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.
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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.
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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.
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Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175.
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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
Michelle Gray, Dyna I Shirasaki, Carlos Cepeda, Véronique M André, Brian Wilburn, Xiao-Hong Lu, Jifang Tao, Irene Yamazaki, Shi-Hua Li, Yi E Sun,[...]. J Neurosci 2008
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Neuropathological classification of Huntington's disease.
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Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.
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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.
Kirupa Sathasivam, Andreas Neueder, Theresa A Gipson, Christian Landles, Agnesska C Benjamin, Marie K Bondulich, Donna L Smith, Richard L M Faull, Raymund A C Roos, David Howland,[...]. Proc Natl Acad Sci U S A 2013
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Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease.
Taneli Heikkinen, Kimmo Lehtimäki, Nina Vartiainen, Jukka Puoliväli, Susan J Hendricks, Jack R Glaser, Amyaouch Bradaia, Kristian Wadel, Chrystelle Touller, Outi Kontkanen,[...]. PLoS One 2012
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Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice.
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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
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Targeting Huntingtin Expression in Patients with Huntington's Disease.
Sarah J Tabrizi, Blair R Leavitt, G Bernhard Landwehrmeyer, Edward J Wild, Carsten Saft, Roger A Barker, Nick F Blair, David Craufurd, Josef Priller, Hugh Rickards,[...]. N Engl J Med 2019
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Neurological abnormalities in a knock-in mouse model of Huntington's disease.
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A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.
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Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism.
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The corticostriatal pathway in Huntington's disease.
Carlos Cepeda, Nanping Wu, Véronique M André, Damian M Cummings, Michael S Levine. Prog Neurobiol 2007
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In Situ Architecture and Cellular Interactions of PolyQ Inclusions.
Felix J B Bäuerlein, Itika Saha, Archana Mishra, Maria Kalemanov, Antonio Martínez-Sánchez, Rüdiger Klein, Irina Dudanova, Mark S Hipp, F Ulrich Hartl, Wolfgang Baumeister,[...]. Cell 2017
Felix J B Bäuerlein, Itika Saha, Archana Mishra, Maria Kalemanov, Antonio Martínez-Sánchez, Rüdiger Klein, Irina Dudanova, Mark S Hipp, F Ulrich Hartl, Wolfgang Baumeister,[...]. Cell 2017
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Polyglutamine Repeats in Neurodegenerative Diseases.
Andrew P Lieberman, Vikram G Shakkottai, Roger L Albin. Annu Rev Pathol 2019
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Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models.
Liliana Menalled, Bassem F El-Khodor, Monica Patry, Mayte Suárez-Fariñas, Samantha J Orenstein, Benjamin Zahasky, Christina Leahy, Vanessa Wheeler, X William Yang, Marcy MacDonald,[...]. Neurobiol Dis 2009
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The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease.
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Trinucleotide repeat length instability and age of onset in Huntington's disease.
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Electrophysiological and morphological changes in striatal spiny neurons in R6/2 Huntington's disease transgenic mice.
G J Klapstein, R S Fisher, H Zanjani, C Cepeda, E S Jokel, M F Chesselet, M S Levine. J Neurophysiol 2001
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N-terminal Huntingtin (Htt) phosphorylation is a molecular switch regulating Htt aggregation, helical conformation, internalization, and nuclear targeting.
Sean M DeGuire, Francesco S Ruggeri, Mohamed-Bilal Fares, Anass Chiki, Urszula Cendrowska, Giovanni Dietler, Hilal A Lashuel. J Biol Chem 2018
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Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology.
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Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.
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Spatiotemporal Proteomic Profiling of Huntington's Disease Inclusions Reveals Widespread Loss of Protein Function.
Fabian Hosp, Sara Gutiérrez-Ángel, Martin H Schaefer, Jürgen Cox, Felix Meissner, Mark S Hipp, F-Ulrich Hartl, Rüdiger Klein, Irina Dudanova, Matthias Mann. Cell Rep 2017
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Choosing an animal model for the study of Huntington's disease.
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Huntingtin Lowering Strategies for Disease Modification in Huntington's Disease.
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Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
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Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
Christian Landles, Kirupa Sathasivam, Andreas Weiss, Ben Woodman, Hilary Moffitt, Steve Finkbeiner, Banghua Sun, Juliette Gafni, Lisa M Ellerby, Yvon Trottier,[...]. J Biol Chem 2010
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RAN Translation in Huntington Disease.
Monica Bañez-Coronel, Fatma Ayhan, Alex D Tarabochia, Tao Zu, Barbara A Perez, Solaleh Khoramian Tusi, Olga Pletnikova, David R Borchelt, Christopher A Ross, Russell L Margolis,[...]. Neuron 2015
Monica Bañez-Coronel, Fatma Ayhan, Alex D Tarabochia, Tao Zu, Barbara A Perez, Solaleh Khoramian Tusi, Olga Pletnikova, David R Borchelt, Christopher A Ross, Russell L Margolis,[...]. Neuron 2015
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The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.
Jia Yi Li, Natalija Popovic, Patrik Brundin. NeuroRx 2005
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The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis.
Tamara Pringsheim, Katie Wiltshire, Lundy Day, Jonathan Dykeman, Thomas Steeves, Nathalie Jette. Mov Disord 2012
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Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin.
Nicholas Stephane Caron, Carly Robyn Desmond, Jianrun Xia, Ray Truant. Proc Natl Acad Sci U S A 2013
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IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.
Leslie Michels Thompson, Charity T Aiken, Linda S Kaltenbach, Namita Agrawal, Katalin Illes, Ali Khoshnan, Marta Martinez-Vincente, Montserrat Arrasate, Jacqueline Gire O'Rourke, Hasan Khashwji,[...]. J Cell Biol 2009
Leslie Michels Thompson, Charity T Aiken, Linda S Kaltenbach, Namita Agrawal, Katalin Illes, Ali Khoshnan, Marta Martinez-Vincente, Montserrat Arrasate, Jacqueline Gire O'Rourke, Hasan Khashwji,[...]. J Cell Biol 2009
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The emerging role of the first 17 amino acids of huntingtin in Huntington's disease.
James R Arndt, Maxmore Chaibva, Justin Legleiter. Biomol Concepts 2015
James R Arndt, Maxmore Chaibva, Justin Legleiter. Biomol Concepts 2015
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The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients.
Andreas Neueder, Christian Landles, Rhia Ghosh, David Howland, Richard H Myers, Richard L M Faull, Sarah J Tabrizi, Gillian P Bates. Sci Rep 2017
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Potential function for the Huntingtin protein as a scaffold for selective autophagy.
Joseph Ochaba, Tamás Lukacsovich, George Csikos, Shuqiu Zheng, Julia Margulis, Lisa Salazar, Kai Mao, Alice L Lau, Sylvia Y Yeung, Sandrine Humbert,[...]. Proc Natl Acad Sci U S A 2014
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6
Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.