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Times Cited: 157
Times Cited: 157
Times Cited
Times Co-cited
Similarity
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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.
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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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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.
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Selective striatal neuronal loss in a YAC128 mouse model of 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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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
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Towards a transgenic model of Huntington's disease in a non-human primate.
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Huntington's disease: from molecular pathogenesis to clinical treatment.
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Neurological abnormalities in a knock-in mouse model of Huntington's disease.
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Choosing an animal model for the study of Huntington's disease.
Mahmoud A Pouladi, A Jennifer Morton, Michael R Hayden. Nat Rev Neurosci 2013
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Huntington disease.
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Molecular mechanisms and potential therapeutical targets in Huntington's disease.
Chiara Zuccato, Marta Valenza, Elena Cattaneo. Physiol Rev 2010
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Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.
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Huntington disease: natural history, biomarkers and prospects for therapeutics.
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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse.
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Network organization of the huntingtin proteomic interactome in mammalian brain.
Dyna I Shirasaki, Erin R Greiner, Ismael Al-Ramahi, Michelle Gray, Pinmanee Boontheung, Daniel H Geschwind, Juan Botas, Giovanni Coppola, Steve Horvath, Joseph A Loo,[...]. Neuron 2012
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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.
Liliana B Menalled, Jessica D Sison, Ioannis Dragatsis, Scott Zeitlin, Marie-Françoise Chesselet. J Comp Neurol 2003
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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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Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.
Alexandre Kuhn, Darlene R Goldstein, Angela Hodges, Andrew D Strand, Thierry Sengstag, Charles Kooperberg, Kristina Becanovic, Mahmoud A Pouladi, Kirupa Sathasivam, Jang-Ho J Cha,[...]. Hum Mol Genet 2007
Alexandre Kuhn, Darlene R Goldstein, Angela Hodges, Andrew D Strand, Thierry Sengstag, Charles Kooperberg, Kristina Becanovic, Mahmoud A Pouladi, Kirupa Sathasivam, Jang-Ho J Cha,[...]. Hum Mol Genet 2007
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Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice.
Xiaofeng Gu, Erin R Greiner, Rakesh Mishra, Ravindra Kodali, Alex Osmand, Steven Finkbeiner, Joan S Steffan, Leslie Michels Thompson, Ronald Wetzel, X William Yang. Neuron 2009
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12
Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs.
Dongshan Yang, Chuan-En Wang, Bentian Zhao, Wei Li, Zhen Ouyang, Zhaoming Liu, Huaqiang Yang, Pei Fan, Ashley O'Neill, Weiwang Gu,[...]. Hum Mol Genet 2010
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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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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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An ovine transgenic Huntington's disease model.
Jessie C Jacobsen, C Simon Bawden, Skye R Rudiger, Clive J McLaughlan, Suzanne J Reid, Henry J Waldvogel, Marcy E MacDonald, James F Gusella, Simon K Walker, Jennifer M Kelly,[...]. Hum Mol Genet 2010
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Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.
R J Carter, L A Lione, T Humby, L Mangiarini, A Mahal, G P Bates, S B Dunnett, A J Morton. J Neurosci 1999
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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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A transgenic minipig model of Huntington's Disease.
Monika Baxa, Marian Hruska-Plochan, Stefan Juhas, Petr Vodicka, Antonin Pavlok, Jana Juhasova, Atsushi Miyanohara, Tetsuya Nejime, Jiri Klima, Monika Macakova,[...]. J Huntingtons Dis 2013
Monika Baxa, Marian Hruska-Plochan, Stefan Juhas, Petr Vodicka, Antonin Pavlok, Jana Juhasova, Atsushi Miyanohara, Tetsuya Nejime, Jiri Klima, Monika Macakova,[...]. J Huntingtons Dis 2013
14
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
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A Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington's Disease.
Sen Yan, Zhuchi Tu, Zhaoming Liu, Nana Fan, Huiming Yang, Su Yang, Weili Yang, Yu Zhao, Zhen Ouyang, Chengdan Lai,[...]. Cell 2018
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10
Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease.
Jeremy M Van Raamsdonk, Jacqueline Pearson, Elizabeth J Slow, Sazzad M Hossain, Blair R Leavitt, Michael R Hayden. J Neurosci 2005
Jeremy M Van Raamsdonk, Jacqueline Pearson, Elizabeth J Slow, Sazzad M Hossain, Blair R Leavitt, Michael R Hayden. J Neurosci 2005
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Transgenic rat model of Huntington's disease.
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Mouse models of Huntington's disease.
Liliana B Menalled, Marie-Françoise Chesselet. Trends Pharmacol Sci 2002
Liliana B Menalled, Marie-Françoise Chesselet. Trends Pharmacol Sci 2002
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Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin.
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Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice.
Mahmoud A Pouladi, Lisa M Stanek, Yuanyun Xie, Sonia Franciosi, Amber L Southwell, Yu Deng, Stefanie Butland, Weining Zhang, Seng H Cheng, Lamya S Shihabuddin,[...]. Hum Mol Genet 2012
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9
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease.
Nan Wang, Michelle Gray, Xiao-Hong Lu, Jeffrey P Cantle, Sandra M Holley, Erin Greiner, Xiaofeng Gu, Dyna Shirasaki, Carlos Cepeda, Yuqing Li,[...]. Nat Med 2014
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Genetic mouse models of Huntington's and Parkinson's diseases: illuminating but imperfect.
Michael S Levine, Carlos Cepeda, Miriam A Hickey, Sheila M Fleming, Marie-Françoise Chesselet. Trends Neurosci 2004
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9
Normal huntingtin function: an alternative approach to Huntington's disease.
Elena Cattaneo, Chiara Zuccato, Marzia Tartari. Nat Rev Neurosci 2005
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8
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death.
Montserrat Arrasate, Siddhartha Mitra, Erik S Schweitzer, Mark R Segal, Steven Finkbeiner. Nature 2004
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8
Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits.
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Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function.
L A Raymond, V M André, C Cepeda, C M Gladding, A J Milnerwood, M S Levine. Neuroscience 2011
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8
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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8
Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors.
Andrea Crotti, Christopher Benner, Bilal E Kerman, David Gosselin, Clotilde Lagier-Tourenne, Chiara Zuccato, Elena Cattaneo, Fred H Gage, Don W Cleveland, Christopher K Glass. Nat Neurosci 2014
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8
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.
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8
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.