Published in J Biol Chem on July 21, 2005
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Ataxin-3 is a multivalent ligand for the parkin Ubl domain. Biochemistry (2013) 0.78
Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models. J Cell Biol (2016) 0.77
Ataxin-3 consolidates the MDC1-dependent DNA double-strand break response by counteracting the SUMO-targeted ubiquitin ligase RNF4. EMBO J (2017) 0.75
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Brain structure in preclinical Huntington's disease. Biol Psychiatry (2005) 2.05
Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet (2010) 1.99
Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol Cell (2005) 1.84
Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia. J Neurosci (2004) 1.71
CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci (2005) 1.57
Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia. J Neurosci (2005) 1.53
Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis. Proc Natl Acad Sci U S A (2002) 1.48
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Toward understanding Machado-Joseph disease. Prog Neurobiol (2011) 1.42
Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration. Curr Opin Genet Dev (2003) 1.41
Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. Mol Cell (2011) 1.37
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiol Dis (2008) 1.28
Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex. J Alzheimers Dis (2012) 1.27
Targeting Alzheimer's disease genes with RNA interference: an efficient strategy for silencing mutant alleles. Nucleic Acids Res (2004) 1.27
The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Hum Mol Genet (2010) 1.21
Molecular medicine for the brain: silencing of disease genes with RNA interference. Lancet Neurol (2004) 1.20
Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration. Hum Mol Genet (2009) 1.18
Obsessive and compulsive symptoms in prediagnosed Huntington's disease. J Clin Psychiatry (2008) 1.17
Balancing act: deubiquitinating enzymes in the nervous system. Trends Neurosci (2011) 1.17
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Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity. J Biol Chem (2007) 1.14
Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117. J Biol Chem (2010) 1.12
Accelerated neurodegeneration through chaperone-mediated oligomerization of tau. J Clin Invest (2013) 1.12
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Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome. PLoS Genet (2010) 1.11
Allele-specific RNAi mitigates phenotypic progression in a transgenic model of Alzheimer's disease. Mol Ther (2009) 1.09
Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. J Neurochem (2004) 1.08
Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit. J Neurosci (2007) 1.06
Diversity in tissue expression, substrate binding, and SCF complex formation for a lectin family of ubiquitin ligases. J Biol Chem (2008) 1.05
The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates. J Biol Chem (2013) 1.03
Toward therapy for DYT1 dystonia: allele-specific silencing of mutant TorsinA. Ann Neurol (2003) 1.00
Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One (2010) 0.99
Technology insight: therapeutic RNA interference--how far from the neurology clinic? Nat Clin Pract Neurol (2007) 0.99
A novel route for F-box protein-mediated ubiquitination links CHIP to glycoprotein quality control. J Biol Chem (2006) 0.99
Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease. J Mol Biol (2004) 0.98
The E3 ubiquitin ligase CHIP and the molecular chaperone Hsc70 form a dynamic, tethered complex. Biochemistry (2013) 0.95
Analysis of the tau-associated proteome reveals that exchange of Hsp70 for Hsp90 is involved in tau degradation. ACS Chem Biol (2012) 0.94
Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice. Mol Ther (2013) 0.94
Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. Brain Pathol (2012) 0.92
Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Mol Ther (2013) 0.91
Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLoS One (2010) 0.91
Physiologic alterations in ataxia: channeling changes into novel therapies. Arch Neurol (2009) 0.90
Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Ann Clin Psychiatry (2008) 0.88
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Early alterations of autophagy in Huntington disease-like mice. Autophagy (2010) 0.87
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JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosis. J Biol Chem (2013) 0.85
RNA interference in neuroscience: progress and challenges. Cell Mol Neurobiol (2005) 0.84
F-box only protein 2 (Fbxo2) regulates amyloid precursor protein levels and processing. J Biol Chem (2014) 0.83
Establishment of a novel fluorescence-based method to evaluate chaperone-mediated autophagy in a single neuron. PLoS One (2012) 0.83
Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1. Dis Model Mech (2013) 0.82
Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels. PLoS One (2010) 0.82
Compromised mitochondrial complex II in models of Machado-Joseph disease. Biochim Biophys Acta (2011) 0.82
Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3. PLoS One (2012) 0.81
Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3. FEBS Open Bio (2013) 0.79
C9orf72-associated FTD/ALS: when less is more. Neuron (2013) 0.77
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Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study. Acta Neuropathol (2012) 0.76
Dangerous liaisons: polyglutamine meets HMGB. Nat Cell Biol (2007) 0.75
Drug discovery: Kill the messenger where it lives. Nature (2012) 0.75
New hope for therapy in neurodegenerative diseases. Cell Res (2013) 0.75
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