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Times Co-cited
Similarity
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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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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
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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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.
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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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A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.
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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.
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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity.
Barrington Burnett, Fusheng Li, Randall N Pittman. Hum Mol Genet 2003
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SCA3: neurological features, pathogenesis and animal models.
Olaf Riess, Udo Rüb, Annalisa Pastore, Peter Bauer, Ludger Schöls. Cerebellum 2008
Olaf Riess, Udo Rüb, Annalisa Pastore, Peter Bauer, Ludger Schöls. Cerebellum 2008
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Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease.
Philipp Koch, Peter Breuer, Michael Peitz, Johannes Jungverdorben, Jaideep Kesavan, Daniel Poppe, Jonas Doerr, Julia Ladewig, Jerome Mertens, Thomas Tüting,[...]. Nature 2011
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Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
Isabel Nascimento-Ferreira, Tiago Santos-Ferreira, Lígia Sousa-Ferreira, Gwennaëlle Auregan, Isabel Onofre, Sandro Alves, Noëlle Dufour, Veronica F Colomer Gould, Arnulf Koeppen, Nicole Déglon,[...]. Brain 2011
Isabel Nascimento-Ferreira, Tiago Santos-Ferreira, Lígia Sousa-Ferreira, Gwennaëlle Auregan, Isabel Onofre, Sandro Alves, Noëlle Dufour, Veronica F Colomer Gould, Arnulf Koeppen, Nicole Déglon,[...]. Brain 2011
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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo.
H Ikeda, M Yamaguchi, S Sugai, Y Aze, S Narumiya, A Kakizuka. Nat Genet 1996
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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 3.
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Heterogeneous intracellular localization and expression of ataxin-3.
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Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
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Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities.
Fusheng Li, Todd Macfarlan, Randall N Pittman, Debabrata Chakravarti. J Biol Chem 2002
Fusheng Li, Todd Macfarlan, Randall N Pittman, Debabrata Chakravarti. J Biol Chem 2002
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Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis.
Ellen W Doss-Pepe, Edward S Stenroos, William G Johnson, Kiran Madura. Mol Cell Biol 2003
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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
Ina Schmitt, Marion Linden, Hassan Khazneh, Bernd O Evert, Peter Breuer, Thomas Klockgether, Ullrich Wuellner. Biochem Biophys Res Commun 2007
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Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.
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Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.
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Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3.
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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
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Correlation between CAG repeat length and clinical features in Machado-Joseph disease.
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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
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Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3.
Annette Haacke, F Ulrich Hartl, Peter Breuer. J Biol Chem 2007
Annette Haacke, F Ulrich Hartl, Peter Breuer. J Biol Chem 2007
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Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease.
Ana T Simões, Nélio Gonçalves, Arnulf Koeppen, Nicole Déglon, Sebastian Kügler, Carlos Bandeira Duarte, Luís Pereira de Almeida. Brain 2012
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Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions.
Thorsten Schmidt, Katrin S Lindenberg, Antje Krebs, Ludger Schöls, Franco Laccone, Jochen Herms, Martin Rechsteiner, Olaf Riess, G Bernhard Landwehrmeyer. Ann Neurol 2002
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CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3.
Thorsten Mueller, Peter Breuer, Ina Schmitt, Jochen Walter, Bernd O Evert, Ullrich Wüllner. Hum Mol Genet 2009
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Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract.
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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
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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
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The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.
Barrington G Burnett, Randall N Pittman. Proc Natl Acad Sci U S A 2005
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Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways.
Yaohui Chai, Sarah Shoesmith Berke, Robert E Cohen, Henry L Paulson. J Biol Chem 2004
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Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.
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Identification and functional dissection of localization signals within ataxin-3.
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Axonal inclusions in spinocerebellar ataxia type 3.
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Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).
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Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation.
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Machado-Joseph disease gene products carrying different carboxyl termini.
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Udo Rüb, Ewout R Brunt, Thomas Deller. Curr Opin Neurol 2008
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Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3.
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The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability.
Thomas M Durcan, Maria Kontogiannea, Thorhildur Thorarinsdottir, Lara Fallon, Aislinn J Williams, Ana Djarmati, Tadeu Fantaneanu, Henry L Paulson, Edward A Fon. Hum Mol Genet 2011
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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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Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation.
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Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates.
Xiaoyan Zhong, Randall N Pittman. Hum Mol Genet 2006
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Improvement in the molecular diagnosis of Machado-Joseph disease.
P Maciel, M C Costa, A Ferro, M Rousseau, C S Santos, C Gaspar, J Barros, G A Rouleau, P Coutinho, J Sequeiros. Arch Neurol 2001
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Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.
Sokol V Todi, Brett J Winborn, K Matthew Scaglione, Jessica R Blount, Sue M Travis, Henry L Paulson. EMBO J 2009
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Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3.
Bernd O Evert, Ina R Vogt, Ana M Vieira-Saecker, Lucia Ozimek, Rob A I de Vos, Ewout R P Brunt, Thomas Klockgether, Ullrich Wüllner. J Neuropathol Exp Neurol 2003
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Toward understanding Machado-Joseph disease.
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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.