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Times Co-cited
Similarity
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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.
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An-Hsun Chou, Tu-Hsueh Yeh, Pin Ouyang, Ying-Ling Chen, Si-Ying Chen, Hung-Li Wang. Neurobiol Dis 2008
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Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease.
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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain.
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Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.
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Polyglutamine tracts regulate beclin 1-dependent autophagy.
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Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3.
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Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease.
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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
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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity.
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YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.
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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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Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study.
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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
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Scale for the assessment and rating of ataxia: development of a new clinical scale.
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Machado-Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy.
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The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.
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Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.
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Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence.
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Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease.
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DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases.
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The gene for Machado-Joseph disease maps to human chromosome 14q.
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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3.
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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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Serotonergic signalling suppresses ataxin 3 aggregation and neurotoxicity in animal models of Machado-Joseph disease.
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Spinocerebellar ataxia.
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Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates 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.