Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Hirokazu Hirai, Nicole Déglon, Luís Pereira de Almeida. PLoS One 2013
Times Cited: 84
Times Cited: 84
Times Cited
Times Co-cited
Similarity
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
52
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
48
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
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
47
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
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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
39
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
28
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
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
26
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
30
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
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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
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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
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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
25
Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: removal of the CAG containing exon.
Melvin M Evers, Hoang-Dai Tran, Ioannis Zalachoras, Barry A Pepers, Onno C Meijer, Johan T den Dunnen, Gert-Jan B van Ommen, Annemieke Aartsma-Rus, Willeke M C van Roon-Mom. Neurobiol Dis 2013
Melvin M Evers, Hoang-Dai Tran, Ioannis Zalachoras, Barry A Pepers, Onno C Meijer, Johan T den Dunnen, Gert-Jan B van Ommen, Annemieke Aartsma-Rus, Willeke M C van Roon-Mom. Neurobiol Dis 2013
36
Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease.
Isabel Nascimento-Ferreira, Clévio Nóbrega, Ana Vasconcelos-Ferreira, Isabel Onofre, David Albuquerque, Célia Aveleira, Hirokazu Hirai, Nicole Déglon, Luís Pereira de Almeida. Brain 2013
Isabel Nascimento-Ferreira, Clévio Nóbrega, Ana Vasconcelos-Ferreira, Isabel Onofre, David Albuquerque, Célia Aveleira, Hirokazu Hirai, Nicole Déglon, Luís Pereira de Almeida. Brain 2013
26
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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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
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
21
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
21
RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease.
Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Nicole Déglon, Luís Pereira de Almeida. PLoS One 2014
Clévio Nóbrega, Isabel Nascimento-Ferreira, Isabel Onofre, David Albuquerque, Nicole Déglon, Luís Pereira de Almeida. PLoS One 2014
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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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20
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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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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20
Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.
Takashi Torashima, Chiho Koyama, Akira Iizuka, Kazuhiro Mitsumura, Kiyohiko Takayama, Shigeru Yanagi, Miho Oue, Haruyasu Yamaguchi, Hirokazu Hirai. EMBO Rep 2008
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20
Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs.
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Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.
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17
Toward understanding Machado-Joseph disease.
Maria do Carmo Costa, Henry L Paulson. Prog Neurobiol 2012
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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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17
An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.
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17
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
17
Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.
Anabela Silva-Fernandes, Sara Duarte-Silva, Andreia Neves-Carvalho, Marina Amorim, Carina Soares-Cunha, Pedro Oliveira, Kenneth Thirstrup, Andreia Teixeira-Castro, Patrícia Maciel. Neurotherapeutics 2014
Anabela Silva-Fernandes, Sara Duarte-Silva, Andreia Neves-Carvalho, Marina Amorim, Carina Soares-Cunha, Pedro Oliveira, Kenneth Thirstrup, Andreia Teixeira-Castro, Patrícia Maciel. Neurotherapeutics 2014
21
Transplantation of cerebellar neural stem cells improves motor coordination and neuropathology in Machado-Joseph disease mice.
Liliana S Mendonça, Clévio Nóbrega, Hirokazu Hirai, Brian K Kaspar, Luís Pereira de Almeida. Brain 2015
Liliana S Mendonça, Clévio Nóbrega, Hirokazu Hirai, Brian K Kaspar, Luís Pereira de Almeida. Brain 2015
26
Intravenous administration of brain-targeted stable nucleic acid lipid particles alleviates Machado-Joseph disease neurological phenotype.
Mariana Conceição, Liliana Mendonça, Clévio Nóbrega, Célia Gomes, Pedro Costa, Hirokazu Hirai, João Nuno Moreira, Maria C Lima, N Manjunath, Luís Pereira de Almeida. Biomaterials 2016
Mariana Conceição, Liliana Mendonça, Clévio Nóbrega, Célia Gomes, Pedro Costa, Hirokazu Hirai, João Nuno Moreira, Maria C Lima, N Manjunath, Luís Pereira de Almeida. Biomaterials 2016
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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
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Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3.
Vikram G Shakkottai, Maria do Carmo Costa, James M Dell'Orco, Ananthakrishnan Sankaranarayanan, Heike Wulff, Henry L Paulson. J Neurosci 2011
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16
Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway.
Janete Cunha-Santos, Joana Duarte-Neves, Vitor Carmona, Leonard Guarente, Luís Pereira de Almeida, Cláudia Cavadas. Nat Commun 2016
Janete Cunha-Santos, Joana Duarte-Neves, Vitor Carmona, Leonard Guarente, Luís Pereira de Almeida, Cláudia Cavadas. Nat Commun 2016
22
Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice.
Hayley S McLoughlin, Lauren R Moore, Ravi Chopra, Robert Komlo, Megan McKenzie, Kate G Blumenstein, Hien Zhao, Holly B Kordasiewicz, Vikram G Shakkottai, Henry L Paulson. Ann Neurol 2018
Hayley S McLoughlin, Lauren R Moore, Ravi Chopra, Robert Komlo, Megan McKenzie, Kate G Blumenstein, Hien Zhao, Holly B Kordasiewicz, Vikram G Shakkottai, Henry L Paulson. Ann Neurol 2018
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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo.
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Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study.
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Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3.
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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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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
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Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.
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14
HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3.
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Improvement in the molecular diagnosis of Machado-Joseph disease.
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14
H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse.
Hung-Li Wang, Su-Huei Hu, An-Hsun Chou, Shang-Seng Wang, Yi-Hsin Weng, Tu-Hsueh Yeh. Neuropharmacology 2013
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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.
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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.
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Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.
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Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.
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Serotonergic signalling suppresses ataxin 3 aggregation and neurotoxicity in animal models of Machado-Joseph disease.
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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.