Thorsten Mueller, Peter Breuer, Ina Schmitt, Jochen Walter, Bernd O Evert, Ullrich Wüllner. Hum Mol Genet 2009
Times Cited: 69
Times Cited: 69
Times Cited
Times Co-cited
Similarity
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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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
37
Phosphorylation of ataxin-3 by glycogen synthase kinase 3beta at serine 256 regulates the aggregation of ataxin-3.
Erkang Fei, Nali Jia, Tao Zhang, Xiaochuan Ma, Hongfeng Wang, Chao Liu, Wei Zhang, Lili Ding, Nobuyuki Nukina, Guanghui Wang. Biochem Biophys Res Commun 2007
Erkang Fei, Nali Jia, Tao Zhang, Xiaochuan Ma, Hongfeng Wang, Chao Liu, Wei Zhang, Lili Ding, Nobuyuki Nukina, Guanghui Wang. Biochem Biophys Res Commun 2007
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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
36
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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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
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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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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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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
30
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
28
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
28
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
27
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
27
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
27
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
26
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
24
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
24
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
24
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
25
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
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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
H Ikeda, M Yamaguchi, S Sugai, Y Aze, S Narumiya, A Kakizuka. Nat Genet 1996
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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
23
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
Nihar Ranjan Jana, Priyanka Dikshit, Anand Goswami, Svetlana Kotliarova, Shigeo Murata, Keiji Tanaka, Nobuyuki Nukina. J Biol Chem 2005
23
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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Nucleocytoplasmic shuttling activity of ataxin-3.
Sandra Macedo-Ribeiro, Luísa Cortes, Patrícia Maciel, Ana Luísa Carvalho. PLoS One 2009
Sandra Macedo-Ribeiro, Luísa Cortes, Patrícia Maciel, Ana Luísa Carvalho. PLoS One 2009
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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
25
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
Masaki Matsumoto, Masayoshi Yada, Shigetsugu Hatakeyama, Hiroshi Ishimoto, Teiichi Tanimura, Shoji Tsuji, Akira Kakizuka, Masatoshi Kitagawa, Keiichi I Nakayama. EMBO J 2004
23
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
23
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
21
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
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
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
21
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
34
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
21
Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models.
Carlos A Matos, Clévio Nóbrega, Susana R Louros, Bruno Almeida, Elisabete Ferreiro, Jorge Valero, Luís Pereira de Almeida, Sandra Macedo-Ribeiro, Ana Luísa Carvalho. J Cell Biol 2016
Carlos A Matos, Clévio Nóbrega, Susana R Louros, Bruno Almeida, Elisabete Ferreiro, Jorge Valero, Luís Pereira de Almeida, Sandra Macedo-Ribeiro, Ana Luísa Carvalho. J Cell Biol 2016
62
SUMOylation of the brain-predominant Ataxin-3 isoform modulates its interaction with p97.
Bruno Almeida, Isabel A Abreu, Carlos A Matos, Joana S Fraga, Sara Fernandes, Maria G Macedo, Ricardo Gutiérrez-Gallego, Pedro José Barbosa Pereira, Ana Luísa Carvalho, Sandra Macedo-Ribeiro. Biochim Biophys Acta 2015
Bruno Almeida, Isabel A Abreu, Carlos A Matos, Joana S Fraga, Sara Fernandes, Maria G Macedo, Ricardo Gutiérrez-Gallego, Pedro José Barbosa Pereira, Ana Luísa Carvalho, Sandra Macedo-Ribeiro. Biochim Biophys Acta 2015
57
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
20
The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step.
Andrew M Ellisdon, Bronwen Thomas, Stephen P Bottomley. J Biol Chem 2006
Andrew M Ellisdon, Bronwen Thomas, Stephen P Bottomley. J Biol Chem 2006
20
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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Casein kinase 2 interacts with and phosphorylates ataxin-3.
Rui-Song Tao, Er-Kang Fei, Zheng Ying, Hong-Feng Wang, Guang-Hui Wang. Neurosci Bull 2008
Rui-Song Tao, Er-Kang Fei, Zheng Ying, Hong-Feng Wang, Guang-Hui Wang. Neurosci Bull 2008
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Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B.
G Wang, N Sawai, S Kotliarova, I Kanazawa, N Nukina. Hum Mol Genet 2000
G Wang, N Sawai, S Kotliarova, I Kanazawa, N Nukina. Hum Mol Genet 2000
18
Study of subcellular localization and proteolysis of ataxin-3.
Chiara Pozzi, Marco Valtorta, Gabriella Tedeschi, Elena Galbusera, Valentina Pastori, Alessandra Bigi, Simona Nonnis, Eleonora Grassi, Paola Fusi. Neurobiol Dis 2008
Chiara Pozzi, Marco Valtorta, Gabriella Tedeschi, Elena Galbusera, Valentina Pastori, Alessandra Bigi, Simona Nonnis, Eleonora Grassi, Paola Fusi. Neurobiol Dis 2008
28
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
18
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
18
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
18
Nuclear aggregation of polyglutamine-expanded ataxin-3: fragments escape the cytoplasmic quality control.
Peter Breuer, Annette Haacke, Bernd O Evert, Ullrich Wüllner. J Biol Chem 2010
Peter Breuer, Annette Haacke, Bernd O Evert, Ullrich Wüllner. J Biol Chem 2010
50
Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.
Ginny Marie Harris, Katerina Dodelzon, Lijie Gong, Pedro Gonzalez-Alegre, Henry L Paulson. PLoS One 2010
Ginny Marie Harris, Katerina Dodelzon, Lijie Gong, Pedro Gonzalez-Alegre, Henry L Paulson. PLoS One 2010
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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
18
SUMO-1 modification on K166 of polyQ-expanded ataxin-3 strengthens its stability and increases its cytotoxicity.
Ya-Fang Zhou, Shu-Sheng Liao, Ying-Ying Luo, Jian-Guang Tang, Jun-Ling Wang, Li-Fang Lei, Jing-Wei Chi, Juan Du, Hong Jiang, Kun Xia,[...]. PLoS One 2013
Ya-Fang Zhou, Shu-Sheng Liao, Ying-Ying Luo, Jian-Guang Tang, Jun-Ling Wang, Li-Fang Lei, Jing-Wei Chi, Juan Du, Hong Jiang, Kun Xia,[...]. PLoS One 2013
48
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.