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Times Cited: 75
Times Cited: 75
Times Cited
Times Co-cited
Similarity
Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.
Ulrike Bichelmeier, Thorsten Schmidt, Jeannette Hübener, Jana Boy, Lukas Rüttiger, Karina Häbig, Sven Poths, Michael Bonin, Marlies Knipper, Werner J Schmidt,[...]. J Neurosci 2007
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CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
Y Kawaguchi, T Okamoto, M Taniwaki, M Aizawa, M Inoue, S Katayama, H Kawakami, S Nakamura, M Nishimura, I Akiguchi. Nat Genet 1994
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A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.
Daniel Goti, Scott M Katzen, Jesse Mez, Noam Kurtis, Jennifer Kiluk, Lea Ben-Haïem, Nancy A Jenkins, Neal G Copeland, Akira Kakizuka, Alan H Sharp,[...]. J Neurosci 2004
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YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.
Cemal K Cemal, Christopher J Carroll, Lorraine Lawrence, Margaret B Lowrie, Piers Ruddle, Sahar Al-Mahdawi, Rosalind H M King, Mark A Pook, Clare Huxley, Susan Chamberlain. Hum Mol Genet 2002
Cemal K Cemal, Christopher J Carroll, Lorraine Lawrence, Margaret B Lowrie, Piers Ruddle, Sahar Al-Mahdawi, Rosalind H M King, Mark A Pook, Clare Huxley, Susan Chamberlain. Hum Mol Genet 2002
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A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.
Jana Boy, Thorsten Schmidt, Ulrike Schumann, Ute Grasshoff, Samy Unser, Carsten Holzmann, Ina Schmitt, Tim Karl, Franco Laccone, Hartwig Wolburg,[...]. Neurobiol Dis 2010
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Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation.
An-Hsun Chou, Tu-Hsueh Yeh, Pin Ouyang, Ying-Ling Chen, Si-Ying Chen, Hung-Li Wang. Neurobiol Dis 2008
An-Hsun Chou, Tu-Hsueh Yeh, Pin Ouyang, Ying-Ling Chen, Si-Ying Chen, Hung-Li Wang. Neurobiol Dis 2008
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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo.
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Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products.
Anabela Silva-Fernandes, Maria do Carmo Costa, Sara Duarte-Silva, Pedro Oliveira, Claudia M Botelho, Luís Martins, José António Mariz, Tiago Ferreira, Filipa Ribeiro, Margarida Correia-Neves,[...]. Neurobiol Dis 2010
Anabela Silva-Fernandes, Maria do Carmo Costa, Sara Duarte-Silva, Pedro Oliveira, Claudia M Botelho, Luís Martins, José António Mariz, Tiago Ferreira, Filipa Ribeiro, Margarida Correia-Neves,[...]. Neurobiol Dis 2010
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Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination.
Ina Schmitt, Marion Linden, Hassan Khazneh, Bernd O Evert, Peter Breuer, Thomas Klockgether, Ullrich Wuellner. Biochem Biophys Res Commun 2007
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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3.
Xi Chen, Tie-Shan Tang, Huiping Tu, Omar Nelson, Mark Pook, Robert Hammer, Nobuyuki Nukina, Ilya Bezprozvanny. J Neurosci 2008
Xi Chen, Tie-Shan Tang, Huiping Tu, Omar Nelson, Mark Pook, Robert Hammer, Nobuyuki Nukina, Ilya Bezprozvanny. J Neurosci 2008
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An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.
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Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3?
Sandro Alves, Isabel Nascimento-Ferreira, Noëlle Dufour, Raymonde Hassig, Gwennaëlle Auregan, Clévio Nóbrega, Emmanuel Brouillet, Philippe Hantraye, Maria C Pedroso de Lima, Nicole Déglon,[...]. Hum Mol Genet 2010
Sandro Alves, Isabel Nascimento-Ferreira, Noëlle Dufour, Raymonde Hassig, Gwennaëlle Auregan, Clévio Nóbrega, Emmanuel Brouillet, Philippe Hantraye, Maria C Pedroso de Lima, Nicole Déglon,[...]. Hum Mol Genet 2010
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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
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Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.
Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Hirokazu Hirai, Nicole Déglon, Luís Pereira de Almeida. PLoS One 2013
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SCA3: neurological features, pathogenesis and animal models.
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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.
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Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease.
Sandro Alves, Isabel Nascimento-Ferreira, Gwennaëlle Auregan, Raymonde Hassig, Noëlle Dufour, Emmanuel Brouillet, Maria C Pedroso de Lima, Philippe Hantraye, Luís Pereira de Almeida, Nicole Déglon. PLoS One 2008
Sandro Alves, Isabel Nascimento-Ferreira, Gwennaëlle Auregan, Raymonde Hassig, Noëlle Dufour, Emmanuel Brouillet, Maria C Pedroso de Lima, Philippe Hantraye, Luís Pereira de Almeida, Nicole Déglon. PLoS One 2008
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Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice.
Edgardo Rodríguez-Lebrón, Maria do Carmo Costa, Katiuska Luna-Cancalon, Therese M Peron, Svetlana Fischer, Ryan L Boudreau, Beverly L Davidson, Henry L Paulson. Mol Ther 2013
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Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease.
Sandro Alves, Etienne Régulier, Isabel Nascimento-Ferreira, Raymonde Hassig, Noelle Dufour, Arnulf Koeppen, Ana Luísa Carvalho, Sérgio Simões, Maria C Pedroso de Lima, Emmanuel Brouillet,[...]. Hum Mol Genet 2008
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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity.
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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.
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Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease.
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Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.
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Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human.
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HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3.
An-Hsun Chou, Si-Ying Chen, Tu-Hsueh Yeh, Yi-Hsin Weng, Hung-Li Wang. Neurobiol Dis 2011
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Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.
Takashi Torashima, Chiho Koyama, Akira Iizuka, Kazuhiro Mitsumura, Kiyohiko Takayama, Shigeru Yanagi, Miho Oue, Haruyasu Yamaguchi, Hirokazu Hirai. EMBO Rep 2008
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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.
H L Paulson, S S Das, P B Crino, M K Perez, S C Patel, D Gotsdiner, K H Fischbeck, R N Pittman. Ann Neurol 1997
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Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.
John M Warrick, Lance M Morabito, Julide Bilen, Beth Gordesky-Gold, Lynn Z Faust, Henry L Paulson, Nancy M Bonini. Mol Cell 2005
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RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.
Haibin Xia, Qinwen Mao, Steven L Eliason, Scott Q Harper, Inês H Martins, Harry T Orr, Henry L Paulson, Linda Yang, Robert M Kotin, Beverly L Davidson. Nat Med 2004
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Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3.
Vikram G Shakkottai, Maria do Carmo Costa, James M Dell'Orco, Ananthakrishnan Sankaranarayanan, Heike Wulff, Henry L Paulson. J Neurosci 2011
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Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease.
Maria do Carmo Costa, Katiuska Luna-Cancalon, Svetlana Fischer, Naila S Ashraf, Michelle Ouyang, Rahil M Dharia, Lucas Martin-Fishman, Yemen Yang, Vikram G Shakkottai, Beverly L Davidson,[...]. Mol Ther 2013
Maria do Carmo Costa, Katiuska Luna-Cancalon, Svetlana Fischer, Naila S Ashraf, Michelle Ouyang, Rahil M Dharia, Lucas Martin-Fishman, Yemen Yang, Vikram G Shakkottai, Beverly L Davidson,[...]. Mol Ther 2013
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Allele-specific silencing of dominant disease genes.
Victor M Miller, Haibin Xia, Ginger L Marrs, Cynthia M Gouvion, Gloria Lee, Beverly L Davidson, Henry L Paulson. Proc Natl Acad Sci U S A 2003
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The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.
Brett J Winborn, Sue M Travis, Sokol V Todi, K Matthew Scaglione, Ping Xu, Aislinn J Williams, Robert E Cohen, Junmin Peng, Henry L Paulson. J Biol Chem 2008
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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.
Jing Liu, Tie-Shan Tang, Huiping Tu, Omar Nelson, Emily Herndon, Duong P Huynh, Stefan M Pulst, Ilya Bezprozvanny. J Neurosci 2009
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A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD.
Pawel M Switonski, Wojciech J Szlachcic, Wlodzimierz J Krzyzosiak, Maciej Figiel. Neurobiol Dis 2015
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Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
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Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3.
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Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila.
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A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.
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Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
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Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.
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Inflammatory genes are upregulated in expanded ataxin-3-expressing cell lines and spinocerebellar ataxia type 3 brains.
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Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3.
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Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.
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A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice.
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Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities.
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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.