Ina Schmitt, Marion Linden, Hassan Khazneh, Bernd O Evert, Peter Breuer, Thomas Klockgether, Ullrich Wuellner. Biochem Biophys Res Commun 2007
Times Cited: 104
Times Cited: 104
Times Cited
Times Co-cited
Similarity
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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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
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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
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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
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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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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
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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
Ellen W Doss-Pepe, Edward S Stenroos, William G Johnson, Kiran Madura. Mol Cell Biol 2003
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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
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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
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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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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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Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3.
Ana-João Rodrigues, Giovanni Coppola, Cláudia Santos, Maria do Carmo Costa, Michael Ailion, Jorge Sequeiros, Daniel H Geschwind, Patrícia Maciel. FASEB J 2007
Ana-João Rodrigues, Giovanni Coppola, Cláudia Santos, Maria do Carmo Costa, Michael Ailion, Jorge Sequeiros, Daniel H Geschwind, Patrícia Maciel. FASEB J 2007
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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
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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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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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
28
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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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
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
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Polyglutamine diseases: the special case of ataxin-3 and Machado-Joseph disease.
Carlos A Matos, Sandra de Macedo-Ribeiro, Ana Luísa Carvalho. Prog Neurobiol 2011
Carlos A Matos, Sandra de Macedo-Ribeiro, Ana Luísa Carvalho. Prog Neurobiol 2011
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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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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
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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
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
Yaohui Chai, Sarah Shoesmith Berke, Robert E Cohen, Henry L Paulson. J Biol Chem 2004
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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
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Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain.
Yuxin Mao, Francesca Senic-Matuglia, Pier Paolo Di Fiore, Simona Polo, Michael E Hodsdon, Pietro De Camilli. Proc Natl Acad Sci U S A 2005
Yuxin Mao, Francesca Senic-Matuglia, Pier Paolo Di Fiore, Simona Polo, Michael E Hodsdon, Pietro De Camilli. Proc Natl Acad Sci U S A 2005
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Proteotoxic stress increases nuclear localization of ataxin-3.
Christopher P Reina, Xiaoyan Zhong, Randall N Pittman. Hum Mol Genet 2010
Christopher P Reina, Xiaoyan Zhong, Randall N Pittman. Hum Mol Genet 2010
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Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP.
K Matthew Scaglione, Eszter Zavodszky, Sokol V Todi, Srikanth Patury, Ping Xu, Edgardo Rodríguez-Lebrón, Svetlana Fischer, John Konen, Ana Djarmati, Junmin Peng,[...]. Mol Cell 2011
K Matthew Scaglione, Eszter Zavodszky, Sokol V Todi, Srikanth Patury, Ping Xu, Edgardo Rodríguez-Lebrón, Svetlana Fischer, John Konen, Ana Djarmati, Junmin Peng,[...]. Mol Cell 2011
21
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
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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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Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3.
Sarah J Shoesmith Berke, Yaohui Chai, Ginger L Marrs, Hsiang Wen, Henry L Paulson. J Biol Chem 2005
Sarah J Shoesmith Berke, Yaohui Chai, Ginger L Marrs, Hsiang Wen, Henry L Paulson. J Biol Chem 2005
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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
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Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3.
Giuseppe Nicastro, Sokol V Todi, Ezgi Karaca, Alexandre M J J Bonvin, Henry L Paulson, Annalisa Pastore. PLoS One 2010
Giuseppe Nicastro, Sokol V Todi, Ezgi Karaca, Alexandre M J J Bonvin, Henry L Paulson, Annalisa Pastore. PLoS One 2010
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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
19
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
Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Hirokazu Hirai, Nicole Déglon, Luís Pereira de Almeida. PLoS One 2013
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Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis.
Ludger Schöls, Peter Bauer, Thorsten Schmidt, Thorsten Schulte, Olaf Riess. Lancet Neurol 2004
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Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.
Nihar Ranjan Jana, Priyanka Dikshit, Anand Goswami, Svetlana Kotliarova, Shigeo Murata, Keiji Tanaka, Nobuyuki Nukina. J Biol Chem 2005
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18
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
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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
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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Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.
Janghoo Lim, Juan Crespo-Barreto, Paymaan Jafar-Nejad, Aaron B Bowman, Ronald Richman, David E Hill, Harry T Orr, Huda Y Zoghbi. Nature 2008
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Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs.
Jiaxin Hu, Masayuki Matsui, Keith T Gagnon, Jacob C Schwartz, Sylvie Gabillet, Khalil Arar, Jun Wu, Ilya Bezprozvanny, David R Corey. Nat Biotechnol 2009
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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.
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Autosomal dominant system degeneration in Portuguese families of the Azores Islands. A new genetic disorder involving cerebellar, pyramidal, extrapyramidal and spinal cord motor functions.
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Molecular clearance of ataxin-3 is regulated by a mammalian E4.
Masaki Matsumoto, Masayoshi Yada, Shigetsugu Hatakeyama, Hiroshi Ishimoto, Teiichi Tanimura, Shoji Tsuji, Akira Kakizuka, Masatoshi Kitagawa, Keiichi I Nakayama. EMBO J 2004
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SCA3: neurological features, pathogenesis and animal models.
Olaf Riess, Udo Rüb, Annalisa Pastore, Peter Bauer, Ludger Schöls. Cerebellum 2008
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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
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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo.
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Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity.
Sokol V Todi, Mario N Laco, Brett J Winborn, Sue M Travis, Hsiang M Wen, Henry L Paulson. J Biol Chem 2007
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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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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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The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition.
Giuseppe Nicastro, Rajesh P Menon, Laura Masino, Philip P Knowles, Neil Q McDonald, Annalisa Pastore. Proc Natl Acad Sci U S A 2005
Giuseppe Nicastro, Rajesh P Menon, Laura Masino, Philip P Knowles, Neil Q McDonald, Annalisa Pastore. Proc Natl Acad Sci U S A 2005
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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.