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
Times Cited: 68
Times Cited: 68
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
42
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
35
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
32
An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.
T Schmidt, G B Landwehrmeyer, I Schmitt, Y Trottier, G Auburger, F Laccone, T Klockgether, M Völpel, J T Epplen, L Schöls,[...]. Brain Pathol 1998
T Schmidt, G B Landwehrmeyer, I Schmitt, Y Trottier, G Auburger, F Laccone, T Klockgether, M Völpel, J T Epplen, L Schöls,[...]. Brain Pathol 1998
30
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
29
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
27
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
25
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
25
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
23
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
23
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
23
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
23
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
23
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease.
Y Chai, S L Koppenhafer, N M Bonini, H L Paulson. J Neurosci 1999
Y Chai, S L Koppenhafer, N M Bonini, H L Paulson. J Neurosci 1999
23
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
23
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
22
Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.
Udo Rüb, Rob A I de Vos, Ewout R Brunt, Tamás Sebestény, Ludger Schöls, Georg Auburger, Jürgen Bohl, Estifanos Ghebremedhin, Kristin Gierga, Kay Seidel,[...]. Brain Pathol 2006
Udo Rüb, Rob A I de Vos, Ewout R Brunt, Tamás Sebestény, Ludger Schöls, Georg Auburger, Jürgen Bohl, Estifanos Ghebremedhin, Kristin Gierga, Kay Seidel,[...]. Brain Pathol 2006
34
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
20
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
20
Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3.
Fiona M Menzies, Jeannette Huebener, Maurizio Renna, Michael Bonin, Olaf Riess, David C Rubinsztein. Brain 2010
Fiona M Menzies, Jeannette Huebener, Maurizio Renna, Michael Bonin, Olaf Riess, David C Rubinsztein. Brain 2010
20
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
20
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
19
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
19
An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis.
Annett Boeddrich, Sébastien Gaumer, Annette Haacke, Nikolay Tzvetkov, Mario Albrecht, Bernd O Evert, Eva C Müller, Rudi Lurz, Peter Breuer, Nancy Schugardt,[...]. EMBO J 2006
Annett Boeddrich, Sébastien Gaumer, Annette Haacke, Nikolay Tzvetkov, Mario Albrecht, Bernd O Evert, Eva C Müller, Rudi Lurz, Peter Breuer, Nancy Schugardt,[...]. EMBO J 2006
19
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
19
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
19
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
19
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
Montserrat Arrasate, Siddhartha Mitra, Erik S Schweitzer, Mark R Segal, Steven Finkbeiner. Nature 2004
19
Polyglutamine neurodegeneration: protein misfolding revisited.
Aislinn J Williams, Henry L Paulson. Trends Neurosci 2008
Aislinn J Williams, Henry L Paulson. Trends Neurosci 2008
19
Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease.
Masahiro Hayashi, Katsuji Kobayashi, Hisakazu Furuta. Psychiatry Clin Neurosci 2003
Masahiro Hayashi, Katsuji Kobayashi, Hisakazu Furuta. Psychiatry Clin Neurosci 2003
48
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
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
19
Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3.
Joonil Jung, Kexiang Xu, Derek Lessing, Nancy M Bonini. Hum Mol Genet 2009
Joonil Jung, Kexiang Xu, Derek Lessing, Nancy M Bonini. Hum Mol Genet 2009
27
Autosomal dominant system degeneration in Portuguese families of the Azores Islands. A new genetic disorder involving cerebellar, pyramidal, extrapyramidal and spinal cord motor functions.
P Coutinho, C Andrade. Neurology 1978
P Coutinho, C Andrade. Neurology 1978
17
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
17
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
24
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
33
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
19
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
An-Hsun Chou, Si-Ying Chen, Tu-Hsueh Yeh, Yi-Hsin Weng, Hung-Li Wang. Neurobiol Dis 2011
17
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
17
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
17
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
17
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
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
17
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
16
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
16
Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3.
Qiuyan Wang, Lianyun Li, Yihong Ye. J Cell Biol 2006
Qiuyan Wang, Lianyun Li, Yihong Ye. J Cell Biol 2006
16
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
16
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
16
Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117.
Sokol V Todi, K Matthew Scaglione, Jessica R Blount, Venkatesha Basrur, Kevin P Conlon, Annalisa Pastore, Kojo Elenitoba-Johnson, Henry L Paulson. J Biol Chem 2010
Sokol V Todi, K Matthew Scaglione, Jessica R Blount, Venkatesha Basrur, Kevin P Conlon, Annalisa Pastore, Kojo Elenitoba-Johnson, Henry L Paulson. J Biol Chem 2010
16
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
16
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
16
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.