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Times Cited: 2282
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Similarity
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M DiFiglia, E Sapp, K O Chase, S W Davies, G P Bates, J P Vonsattel, N Aronin. Science 1997
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Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175.
Liliana B Menalled, Andrea E Kudwa, Sam Miller, Jon Fitzpatrick, Judy Watson-Johnson, Nicole Keating, Melinda Ruiz, Richard Mushlin, William Alosio, Kristi McConnell,[...]. PLoS One 2012
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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.
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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.
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Neuropathological classification of Huntington's disease.
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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice.
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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.
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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.
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Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells.
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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.
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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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The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.
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Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice.
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Peter Langfelder, Jeffrey P Cantle, Doxa Chatzopoulou, Nan Wang, Fuying Gao, Ismael Al-Ramahi, Xiao-Hong Lu, Eliana Marisa Ramos, Karla El-Zein, Yining Zhao,[...]. Nat Neurosci 2016
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Trinucleotide repeat length instability and age of onset in Huntington's disease.
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Regional and cellular gene expression changes in human Huntington's disease brain.
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A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease.
Maria Björkqvist, Edward J Wild, Jenny Thiele, Aurelio Silvestroni, Ralph Andre, Nayana Lahiri, Elsa Raibon, Richard V Lee, Caroline L Benn, Denis Soulet,[...]. J Exp Med 2008
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Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.
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Huntingtin Lowering Strategies for Disease Modification in Huntington's Disease.
Sarah J Tabrizi, Rhia Ghosh, Blair R Leavitt. Neuron 2019
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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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Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease.
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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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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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Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology.
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Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
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Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease.
Marta Martinez-Vicente, Zsolt Talloczy, Esther Wong, Guomei Tang, Hiroshi Koga, Susmita Kaushik, Rosa de Vries, Esperanza Arias, Spike Harris, David Sulzer,[...]. Nat Neurosci 2010
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Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain.
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DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases.
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Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches.
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Preferential loss of striato-external pallidal projection neurons in presymptomatic 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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Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates.
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CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.
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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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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
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Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release.
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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.