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Times Cited: 232
Times Cited
Times Co-cited
Similarity
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
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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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Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3.
Annette Haacke, F Ulrich Hartl, Peter Breuer. J Biol Chem 2007
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Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors.
Kazutoshi Takahashi, Shinya Yamanaka. Cell 2006
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Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
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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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Toward understanding Machado-Joseph disease.
Maria do Carmo Costa, Henry L Paulson. Prog Neurobiol 2012
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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
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Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado-Joseph disease.
Ana Teresa Simões, Nélio Gonçalves, Rui Jorge Nobre, Carlos Bandeira Duarte, Luís Pereira de Almeida. Hum Mol Genet 2014
Ana Teresa Simões, Nélio Gonçalves, Rui Jorge Nobre, Carlos Bandeira Duarte, Luís Pereira de Almeida. Hum Mol Genet 2014
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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
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Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).
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Polyglutamine tracts regulate beclin 1-dependent autophagy.
Avraham Ashkenazi, Carla F Bento, Thomas Ricketts, Mariella Vicinanza, Farah Siddiqi, Mariana Pavel, Ferdinando Squitieri, Maarten C Hardenberg, Sara Imarisio, Fiona M Menzies,[...]. Nature 2017
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CRISPR/Cas9-Targeted Deletion of Polyglutamine in Spinocerebellar Ataxia Type 3-Derived Induced Pluripotent Stem Cells.
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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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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
Barrington Burnett, Fusheng Li, Randall N Pittman. Hum Mol Genet 2003
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Autophagy Promoted the Degradation of Mutant ATXN3 in Neurally Differentiated Spinocerebellar Ataxia-3 Human Induced Pluripotent Stem Cells.
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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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Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.
Udo Rüb, Ludger Schöls, Henry Paulson, Georg Auburger, Pawel Kermer, Joanna C Jen, Kay Seidel, Horst-Werner Korf, Thomas Deller. Prog Neurobiol 2013
Udo Rüb, Ludger Schöls, Henry Paulson, Georg Auburger, Pawel Kermer, Joanna C Jen, Kay Seidel, Horst-Werner Korf, Thomas Deller. Prog Neurobiol 2013
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Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes.
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Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease.
Vitor Carmona, Janete Cunha-Santos, Isabel Onofre, Ana Teresa Simões, Udaya Vijayakumar, Beverly L Davidson, Luís Pereira de Almeida. Mol Ther 2017
Vitor Carmona, Janete Cunha-Santos, Isabel Onofre, Ana Teresa Simões, Udaya Vijayakumar, Beverly L Davidson, Luís Pereira de Almeida. Mol Ther 2017
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Induced pluripotent stem cell - derived neurons for the study of spinocerebellar ataxia type 3.
Susanne K Hansen, Tina C Stummann, Helena Borland, Lis F Hasholt, Zeynep Tümer, Jørgen E Nielsen, Mikkel A Rasmussen, Troels T Nielsen, Justus C A Daechsel, Karina Fog,[...]. Stem Cell Res 2016
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Highly efficient neural conversion of human ES and iPS cells by dual inhibition of SMAD signaling.
Stuart M Chambers, Christopher A Fasano, Eirini P Papapetrou, Mark Tomishima, Michel Sadelain, Lorenz Studer. Nat Biotechnol 2009
Stuart M Chambers, Christopher A Fasano, Eirini P Papapetrou, Mark Tomishima, Michel Sadelain, Lorenz Studer. Nat Biotechnol 2009
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Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells.
Mahru C An, Ningzhe Zhang, Gary Scott, Daniel Montoro, Tobias Wittkop, Sean Mooney, Simon Melov, Lisa M Ellerby. Cell Stem Cell 2012
Mahru C An, Ningzhe Zhang, Gary Scott, Daniel Montoro, Tobias Wittkop, Sean Mooney, Simon Melov, Lisa M Ellerby. Cell Stem Cell 2012
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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
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
13
Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation.
Bernd O Evert, Julieta Araujo, Ana M Vieira-Saecker, Rob A I de Vos, Sigrid Harendza, Thomas Klockgether, Ullrich Wüllner. J Neurosci 2006
Bernd O Evert, Julieta Araujo, Ana M Vieira-Saecker, Rob A I de Vos, Sigrid Harendza, Thomas Klockgether, Ullrich Wüllner. J Neurosci 2006
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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
13
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
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
16
A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3.
Jonasz J Weber, Matthias Golla, Giambattista Guaitoli, Pimthanya Wanichawan, Stefanie N Hayer, Stefan Hauser, Ann-Christin Krahl, Maike Nagel, Sebastian Samer, Eleonora Aronica,[...]. Brain 2017
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Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease.
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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
18
Calpain Inhibition Is Protective in Machado-Joseph Disease Zebrafish Due to Induction of Autophagy.
Maxinne Watchon, Kristy C Yuan, Nick Mackovski, Adam J Svahn, Nicholas J Cole, Claire Goldsbury, Silke Rinkwitz, Thomas S Becker, Garth A Nicholson, Angela S Laird. J Neurosci 2017
Maxinne Watchon, Kristy C Yuan, Nick Mackovski, Adam J Svahn, Nicholas J Cole, Claire Goldsbury, Silke Rinkwitz, Thomas S Becker, Garth A Nicholson, Angela S Laird. J Neurosci 2017
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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
Thorsten Mueller, Peter Breuer, Ina Schmitt, Jochen Walter, Bernd O Evert, Ullrich Wüllner. Hum Mol Genet 2009
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Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models.
Lauren R Moore, Gautam Rajpal, Ian T Dillingham, Maya Qutob, Kate G Blumenstein, Danielle Gattis, Gene Hung, Holly B Kordasiewicz, Henry L Paulson, Hayley S McLoughlin. Mol Ther Nucleic Acids 2017
Lauren R Moore, Gautam Rajpal, Ian T Dillingham, Maya Qutob, Kate G Blumenstein, Danielle Gattis, Gene Hung, Holly B Kordasiewicz, Henry L Paulson, Hayley S McLoughlin. Mol Ther Nucleic Acids 2017
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Induced pluripotent stem cells from a spinal muscular atrophy patient.
Allison D Ebert, Junying Yu, Ferrill F Rose, Virginia B Mattis, Christian L Lorson, James A Thomson, Clive N Svendsen. Nature 2009
Allison D Ebert, Junying Yu, Ferrill F Rose, Virginia B Mattis, Christian L Lorson, James A Thomson, Clive N Svendsen. Nature 2009
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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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Identification and functional dissection of localization signals within ataxin-3.
Paul Michel Aloyse Antony, Simone Mäntele, Phillip Mollenkopf, Jana Boy, Ralph H Kehlenbach, Olaf Riess, Thorsten Schmidt. Neurobiol Dis 2009
Paul Michel Aloyse Antony, Simone Mäntele, Phillip Mollenkopf, Jana Boy, Ralph H Kehlenbach, Olaf Riess, Thorsten Schmidt. Neurobiol Dis 2009
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Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3.
Annette Haacke, Sarah A Broadley, Raina Boteva, Nikolay Tzvetkov, F Ulrich Hartl, Peter Breuer. Hum Mol Genet 2006
Annette Haacke, Sarah A Broadley, Raina Boteva, Nikolay Tzvetkov, F Ulrich Hartl, Peter Breuer. Hum Mol Genet 2006
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Axonal inclusions in spinocerebellar ataxia type 3.
Kay Seidel, Wilfred F A den Dunnen, Christian Schultz, Henry Paulson, Stefanie Frank, Rob A de Vos, Ewout R Brunt, Thomas Deller, Harm H Kampinga, Udo Rüb. Acta Neuropathol 2010
Kay Seidel, Wilfred F A den Dunnen, Christian Schultz, Henry Paulson, Stefanie Frank, Rob A de Vos, Ewout R Brunt, Thomas Deller, Harm H Kampinga, Udo Rüb. Acta Neuropathol 2010
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Brain pathology of spinocerebellar ataxias.
Kay Seidel, Sonny Siswanto, Ewout R P Brunt, Wilfred den Dunnen, Horst-Werner Korf, Udo Rüb. Acta Neuropathol 2012
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12
Generation of human-induced pluripotent stem cells to model spinocerebellar ataxia type 2 in vitro.
Guangbin Xia, Katherine Santostefano, Takashi Hamazaki, Jilin Liu, S H Subramony, Naohiro Terada, Tetsuo Ashizawa. J Mol Neurosci 2013
Guangbin Xia, Katherine Santostefano, Takashi Hamazaki, Jilin Liu, S H Subramony, Naohiro Terada, Tetsuo Ashizawa. J Mol Neurosci 2013
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Vulnerability of Purkinje Cells Generated from Spinocerebellar Ataxia Type 6 Patient-Derived iPSCs.
Yoshihito Ishida, Hideshi Kawakami, Hiroyuki Kitajima, Ayaka Nishiyama, Yoshiki Sasai, Haruhisa Inoue, Keiko Muguruma. Cell Rep 2016
Yoshihito Ishida, Hideshi Kawakami, Hiroyuki Kitajima, Ayaka Nishiyama, Yoshiki Sasai, Haruhisa Inoue, Keiko Muguruma. Cell Rep 2016
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Antisense Oligonucleotide-Mediated Removal of the Polyglutamine Repeat in Spinocerebellar Ataxia Type 3 Mice.
Lodewijk J A Toonen, Frank Rigo, Haico van Attikum, Willeke M C van Roon-Mom. Mol Ther Nucleic Acids 2017
Lodewijk J A Toonen, Frank Rigo, Haico van Attikum, Willeke M C van Roon-Mom. Mol Ther Nucleic Acids 2017
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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.
Y Chai, S L Koppenhafer, S J Shoesmith, M K Perez, H L Paulson. Hum Mol Genet 1999
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Polyglutamine spinocerebellar ataxias - from genes to potential treatments.
Henry L Paulson, Vikram G Shakkottai, H Brent Clark, Harry T Orr. Nat Rev Neurosci 2017
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Serotonergic signalling suppresses ataxin 3 aggregation and neurotoxicity in animal models of Machado-Joseph disease.
Andreia Teixeira-Castro, Ana Jalles, Sofia Esteves, Soosung Kang, Liliana da Silva Santos, Anabela Silva-Fernandes, Mário F Neto, Renée M Brielmann, Carlos Bessa, Sara Duarte-Silva,[...]. Brain 2015
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22
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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Genetics, Mechanisms, and Therapeutic Progress in Polyglutamine Spinocerebellar Ataxias.
Ronald A M Buijsen, Lodewijk J A Toonen, Sarah L Gardiner, Willeke M C van Roon-Mom. Neurotherapeutics 2019
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FOXO4-dependent upregulation of superoxide dismutase-2 in response to oxidative stress is impaired in spinocerebellar ataxia type 3.
Julieta Araujo, Peter Breuer, Susanne Dieringer, Sybille Krauss, Stephanie Dorn, Katrin Zimmermann, Alexander Pfeifer, Thomas Klockgether, Ullrich Wuellner, Bernd O Evert. Hum Mol Genet 2011
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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
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
11
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.