T Schmidt, G B Landwehrmeyer, I Schmitt, Y Trottier, G Auburger, F Laccone, T Klockgether, M Völpel, J T Epplen, L Schöls, O Riess. Brain Pathol 1998
Times Cited: 161
Times Cited: 161
Times Cited
Times Co-cited
Similarity
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
H L Paulson, M K Perez, Y Trottier, J Q Trojanowski, S H Subramony, S S Das, P Vig, J L Mandel, K H Fischbeck, R N Pittman. Neuron 1997
H L Paulson, M K Perez, Y Trottier, J Q Trojanowski, S H Subramony, S S Das, P Vig, J L Mandel, K H Fischbeck, R N Pittman. Neuron 1997
60
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
Y Kawaguchi, T Okamoto, M Taniwaki, M Aizawa, M Inoue, S Katayama, H Kawakami, S Nakamura, M Nishimura, I Akiguchi. Nat Genet 1994
57
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
50
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
42
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
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
38
Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.
A Dürr, G Stevanin, G Cancel, C Duyckaerts, N Abbas, O Didierjean, H Chneiweiss, A Benomar, O Lyon-Caen, J Julien,[...]. Ann Neurol 1996
A Dürr, G Stevanin, G Cancel, C Duyckaerts, N Abbas, O Didierjean, H Chneiweiss, A Benomar, O Lyon-Caen, J Julien,[...]. Ann Neurol 1996
36
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
35
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
35
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
Philipp Koch, Peter Breuer, Michael Peitz, Johannes Jungverdorben, Jaideep Kesavan, Daniel Poppe, Jonas Doerr, Julia Ladewig, Jerome Mertens, Thomas Tüting,[...]. Nature 2011
32
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
31
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
H Ikeda, M Yamaguchi, S Sugai, Y Aze, S Narumiya, A Kakizuka. Nat Genet 1996
30
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
30
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
27
Heterogeneous intracellular localization and expression of ataxin-3.
Y Trottier, G Cancel, I An-Gourfinkel, Y Lutz, C Weber, A Brice, E Hirsch, J L Mandel. Neurobiol Dis 1998
Y Trottier, G Cancel, I An-Gourfinkel, Y Lutz, C Weber, A Brice, E Hirsch, J L Mandel. Neurobiol Dis 1998
33
Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
Ludger Schöls, Peter Bauer, Thorsten Schmidt, Thorsten Schulte, Olaf Riess. Lancet Neurol 2004
Ludger Schöls, Peter Bauer, Thorsten Schmidt, Thorsten Schulte, Olaf Riess. Lancet Neurol 2004
26
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
26
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
Ellen W Doss-Pepe, Edward S Stenroos, William G Johnson, Kiran Madura. Mol Cell Biol 2003
25
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
25
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
John M Warrick, Lance M Morabito, Julide Bilen, Beth Gordesky-Gold, Lynn Z Faust, Henry L Paulson, Nancy M Bonini. Mol Cell 2005
24
Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.
Sarah J Shoesmith Berke, Francisca A Flores Schmied, Ewout R Brunt, Lisa M Ellerby, Henry L Paulson. J Neurochem 2004
Sarah J Shoesmith Berke, Francisca A Flores Schmied, Ewout R Brunt, Lisa M Ellerby, Henry L Paulson. J Neurochem 2004
30
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
25
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
24
Correlation between CAG repeat length and clinical features in Machado-Joseph disease.
P Maciel, C Gaspar, A L DeStefano, I Silveira, P Coutinho, J Radvany, D M Dawson, L Sudarsky, J Guimarães, J E Loureiro. Am J Hum Genet 1995
P Maciel, C Gaspar, A L DeStefano, I Silveira, P Coutinho, J Radvany, D M Dawson, L Sudarsky, J Guimarães, J E Loureiro. Am J Hum Genet 1995
24
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
23
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
31
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
Ana T Simões, Nélio Gonçalves, Arnulf Koeppen, Nicole Déglon, Sebastian Kügler, Carlos Bandeira Duarte, Luís Pereira de Almeida. Brain 2012
27
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
Thorsten Schmidt, Katrin S Lindenberg, Antje Krebs, Ludger Schöls, Franco Laccone, Jochen Herms, Martin Rechsteiner, Olaf Riess, G Bernhard Landwehrmeyer. Ann Neurol 2002
22
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
31
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract.
C L Wellington, L M Ellerby, A S Hackam, R L Margolis, M A Trifiro, R Singaraja, K McCutcheon, G S Salvesen, S S Propp, M Bromm,[...]. J Biol Chem 1998
C L Wellington, L M Ellerby, A S Hackam, R L Margolis, M A Trifiro, R Singaraja, K McCutcheon, G S Salvesen, S S Propp, M Bromm,[...]. J Biol Chem 1998
22
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
22
Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
I A Klement, P J Skinner, M D Kaytor, H Yi, S M Hersch, H B Clark, H Y Zoghbi, H T Orr. Cell 1998
I A Klement, P J Skinner, M D Kaytor, H Yi, S M Hersch, H B Clark, H Y Zoghbi, H T Orr. Cell 1998
21
The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.
Barrington G Burnett, Randall N Pittman. Proc Natl Acad Sci U S A 2005
Barrington G Burnett, Randall N Pittman. Proc Natl Acad Sci U S A 2005
21
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
Yaohui Chai, Sarah Shoesmith Berke, Robert E Cohen, Henry L Paulson. J Biol Chem 2004
21
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
Y Chai, S L Koppenhafer, S J Shoesmith, M K Perez, H L Paulson. Hum Mol Genet 1999
21
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
58
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
30
Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).
Jeannette Hübener, Jonasz Jeremiasz Weber, Claudia Richter, Lisa Honold, Andreas Weiss, Fabronia Murad, Peter Breuer, Ullrich Wüllner, Peter Bellstedt, Francois Paquet-Durand,[...]. Hum Mol Genet 2013
Jeannette Hübener, Jonasz Jeremiasz Weber, Claudia Richter, Lisa Honold, Andreas Weiss, Fabronia Murad, Peter Breuer, Ullrich Wüllner, Peter Bellstedt, Francois Paquet-Durand,[...]. Hum Mol Genet 2013
38
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
20
Machado-Joseph disease gene products carrying different carboxyl termini.
J Goto, M Watanabe, Y Ichikawa, S B Yee, N Ihara, K Endo, S Igarashi, Y Takiyama, C Gaspar, P Maciel,[...]. Neurosci Res 1997
J Goto, M Watanabe, Y Ichikawa, S B Yee, N Ihara, K Endo, S Igarashi, Y Takiyama, C Gaspar, P Maciel,[...]. Neurosci Res 1997
32
New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado-Joseph disease).
Udo Rüb, Ewout R Brunt, Thomas Deller. Curr Opin Neurol 2008
Udo Rüb, Ewout R Brunt, Thomas Deller. Curr Opin Neurol 2008
20
Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3.
Jana Boy, Thorsten Schmidt, Hartwig Wolburg, Andreas Mack, Silke Nuber, Martin Böttcher, Ina Schmitt, Carsten Holzmann, Frank Zimmermann, Antonio Servadio,[...]. Hum Mol Genet 2009
Jana Boy, Thorsten Schmidt, Hartwig Wolburg, Andreas Mack, Silke Nuber, Martin Böttcher, Ina Schmitt, Carsten Holzmann, Frank Zimmermann, Antonio Servadio,[...]. Hum Mol Genet 2009
27
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
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
20
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
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
31
Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation.
M K Perez, H L Paulson, S J Pendse, S J Saionz, N M Bonini, R N Pittman. J Cell Biol 1998
M K Perez, H L Paulson, S J Pendse, S J Saionz, N M Bonini, R N Pittman. J Cell Biol 1998
19
Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates.
Xiaoyan Zhong, Randall N Pittman. Hum Mol Genet 2006
Xiaoyan Zhong, Randall N Pittman. Hum Mol Genet 2006
19
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
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
19
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
Sokol V Todi, Brett J Winborn, K Matthew Scaglione, Jessica R Blount, Sue M Travis, Henry L Paulson. EMBO J 2009
18
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
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
29
Toward understanding Machado-Joseph disease.
Maria do Carmo Costa, Henry L Paulson. Prog Neurobiol 2012
Maria do Carmo Costa, Henry L Paulson. Prog Neurobiol 2012
18
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.