Published in Trends Neurosci on September 06, 2008
CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron (2013) 2.94
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med (2013) 2.89
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron (2009) 2.43
RNA-mediated neurodegeneration in repeat expansion disorders. Ann Neurol (2010) 2.30
Heat shock protein 70 (hsp70) as an emerging drug target. J Med Chem (2010) 2.21
Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet (2010) 1.99
Secondary structure of Huntingtin amino-terminal region. Structure (2009) 1.98
Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease. Nature (2011) 1.87
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43. J Biol Chem (2010) 1.47
Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J (2009) 1.46
Neurodegeneration the RNA way. Prog Neurobiol (2011) 1.43
Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain (2012) 1.42
Toward understanding Machado-Joseph disease. Prog Neurobiol (2011) 1.42
Degeneration and repair in central nervous system disease. Nat Med (2010) 1.42
Heat shock protein 90 in neurodegenerative diseases. Mol Neurodegener (2010) 1.38
Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. Mol Cell (2011) 1.37
Examining polyglutamine peptide length: a connection between collapsed conformations and increased aggregation. J Mol Biol (2009) 1.36
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy. Hum Mol Genet (2012) 1.36
Huntington's disease: underlying molecular mechanisms and emerging concepts. Trends Biochem Sci (2013) 1.31
Intercellular (mis)communication in neurodegenerative disease. Neuron (2012) 1.31
Modulation of polyglutamine conformations and dimer formation by the N-terminus of huntingtin. J Mol Biol (2009) 1.30
The spinocerebellar ataxias. J Neuroophthalmol (2009) 1.30
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies. Exp Mol Med (2015) 1.29
RNA Controls PolyQ Protein Phase Transitions. Mol Cell (2015) 1.28
Recent advances in our understanding of neurodegeneration. J Neural Transm (Vienna) (2009) 1.27
The unstable repeats--three evolving faces of neurological disease. Neuron (2013) 1.26
RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature (2013) 1.18
Balancing act: deubiquitinating enzymes in the nervous system. Trends Neurosci (2011) 1.17
Thymopoiesis in mice depends on a Foxn1-positive thymic epithelial cell lineage. Proc Natl Acad Sci U S A (2010) 1.17
Basic mechanisms of neurodegeneration: a critical update. J Cell Mol Med (2010) 1.13
Formation and toxicity of soluble polyglutamine oligomers in living cells. PLoS One (2010) 1.12
The elimination of accumulated and aggregated proteins: a role for aggrephagy in neurodegeneration. Neurobiol Dis (2010) 1.12
Milestones in ataxia. Mov Disord (2011) 1.11
Entrapment of viral capsids in nuclear PML cages is an intrinsic antiviral host defense against varicella-zoster virus. PLoS Pathog (2011) 1.10
Machado-Joseph disease/spinocerebellar ataxia type 3. Handb Clin Neurol (2012) 1.09
Thermodynamics of beta-sheet formation in polyglutamine. Biophys J (2009) 1.07
A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation. PLoS One (2011) 1.05
The SUN protein Mps3 is required for spindle pole body insertion into the nuclear membrane and nuclear envelope homeostasis. PLoS Genet (2011) 1.04
Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias. Cerebellum (2014) 1.03
Heat shock factor 1 ameliorates proteotoxicity in cooperation with the transcription factor NFAT. EMBO J (2010) 1.02
New insights into repeat instability: role of RNA•DNA hybrids. RNA Biol (2010) 1.01
An optimal ubiquitin-proteasome pathway in the nervous system: the role of deubiquitinating enzymes. Front Mol Neurosci (2014) 1.01
Polyglutamine Atrophin provokes neurodegeneration in Drosophila by repressing fat. EMBO J (2011) 1.01
PGC-1α at the intersection of bioenergetics regulation and neuron function: from Huntington's disease to Parkinson's disease and beyond. Prog Neurobiol (2011) 1.00
Proteinopathy-induced neuronal senescence: a hypothesis for brain failure in Alzheimer's and other neurodegenerative diseases. Alzheimers Res Ther (2009) 1.00
Clinical neurogenetics: autosomal dominant spinocerebellar ataxia. Neurol Clin (2013) 0.99
Disease-associated polyglutamine stretches in monomeric huntingtin adopt a compact structure. J Mol Biol (2012) 0.99
Mitochondrial Ca(2+) and neurodegeneration. Cell Calcium (2012) 0.97
POLG1 polyglutamine tract variants associated with Parkinson's disease. Neurosci Lett (2010) 0.96
Ataxin-1 poly(Q)-induced proteotoxic stress and apoptosis are attenuated in neural cells by docosahexaenoic acid-derived neuroprotectin D1. J Biol Chem (2012) 0.96
Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation. Hum Mol Genet (2011) 0.96
The carboxy-terminal fragment of alpha(1A) calcium channel preferentially aggregates in the cytoplasm of human spinocerebellar ataxia type 6 Purkinje cells. Acta Neuropathol (2009) 0.95
The human epilepsy mutation GABRG2(Q390X) causes chronic subunit accumulation and neurodegeneration. Nat Neurosci (2015) 0.95
Dictyostelium discoideum has a highly Q/N-rich proteome and shows an unusual resilience to protein aggregation. Proc Natl Acad Sci U S A (2015) 0.93
RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome. Hum Mol Genet (2015) 0.92
Epigenetic changes and non-coding expanded repeats. Neurobiol Dis (2010) 0.91
Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Mol Ther (2013) 0.91
Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy. Brain (2013) 0.91
An evaluation of oligonucleotide-based therapeutic strategies for polyQ diseases. BMC Mol Biol (2012) 0.91
Oligonucleotide-based strategies to combat polyglutamine diseases. Nucleic Acids Res (2014) 0.90
Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. Nat Commun (2014) 0.90
Drosophila models of proteinopathies: the little fly that could. Curr Pharm Des (2012) 0.89
Trinucleotide repeats: a structural perspective. Front Neurol (2013) 0.89
Motor neuron disease due to neuropathy target esterase mutation: enzyme analysis of fibroblasts from human subjects yields insights into pathogenesis. Toxicol Lett (2010) 0.89
Caring for Machado-Joseph disease: current understanding and how to help patients. Parkinsonism Relat Disord (2009) 0.88
Ubiquitination regulates the neuroprotective function of the deubiquitinase ataxin-3 in vivo. J Biol Chem (2013) 0.88
Repeat-associated non-AUG translation and its impact in neurodegenerative disease. Neurotherapeutics (2014) 0.88
Transcriptional activation of REST by Sp1 in Huntington's disease models. PLoS One (2010) 0.87
Prion propagation by Hsp40 molecular chaperones. Prion (2009) 0.87
Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease. Hum Mol Genet (2015) 0.87
FAT10 protein binds to polyglutamine proteins and modulates their solubility. J Biol Chem (2011) 0.86
Splice isoform-specific suppression of the Cav2.1 variant underlying spinocerebellar ataxia type 6. Neurobiol Dis (2011) 0.86
Nuclear accumulation of polyglutamine disease proteins and neuropathology. Mol Brain (2009) 0.85
SCA8 CAG/CTG expansions, a tale of two TOXICities: a unique or common case? PLoS Genet (2009) 0.85
Epigenetics in nucleotide repeat expansion disorders. Semin Neurol (2012) 0.85
Elongation kinetics of polyglutamine peptide fibrils: a quartz crystal microbalance with dissipation study. J Mol Biol (2012) 0.84
Amyloid-like fibril formation by polyQ proteins: a critical balance between the polyQ length and the constraints imposed by the host protein. PLoS One (2012) 0.84
An aggregation sensing reporter identifies leflunomide and teriflunomide as polyglutamine aggregate inhibitors. Hum Mol Genet (2011) 0.84
Dynamic imaging by fluorescence correlation spectroscopy identifies diverse populations of polyglutamine oligomers formed in vivo. J Biol Chem (2012) 0.84
Aggregation kinetics of interrupted polyglutamine peptides. J Mol Biol (2011) 0.84
Four decades of neurodegenerative disease research: how far we have come! J Neurosci (2009) 0.84
Using membrane-targeted green fluorescent protein to monitor neurotoxic protein-dependent degeneration of Drosophila eyes. J Neurosci Res (2014) 0.83
Amyloid-associated activity contributes to the severity and toxicity of a prion phenotype. Nat Commun (2014) 0.83
Pathology and function of nuclear amyloid. Protein homeostasis matters. Nucleus (2014) 0.83
RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 0.82
The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders. Neurotherapeutics (2014) 0.82
Hsp40 gene therapy exerts therapeutic effects on polyglutamine disease mice via a non-cell autonomous mechanism. PLoS One (2012) 0.81
RAN translation-What makes it run? Brain Res (2016) 0.81
The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease. J Huntingtons Dis (2013) 0.81
High-throughput screen of natural product extracts in a yeast model of polyglutamine proteotoxicity. Chem Biol Drug Des (2014) 0.80
Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy. Brain (2014) 0.80
Mutation in E1, the ubiquitin activating enzyme, reduces Drosophila lifespan and results in motor impairment. PLoS One (2013) 0.80
Transcriptome sequencing reveals aberrant alternative splicing in Huntington's disease. Hum Mol Genet (2016) 0.78
Real-time imaging of Huntingtin aggregates diverting target search and gene transcription. Elife (2016) 0.78
Neuropathology and therapeutic intervention in spinal and bulbar muscular atrophy. Int J Mol Sci (2009) 0.78
The Social Amoeba Dictyostelium discoideum Is Highly Resistant to Polyglutamine Aggregation. J Biol Chem (2015) 0.78
Inhibition of protein misfolding/aggregation using polyglutamine binding peptide QBP1 as a therapy for the polyglutamine diseases. Neurotherapeutics (2013) 0.77
The Aggregation Inhibitor Peptide QBP1 as a Therapeutic Molecule for the Polyglutamine Neurodegenerative Diseases. J Amino Acids (2011) 0.77
Early retinal function deficit without prominent morphological changes in the R6/2 mouse model of Huntington's disease. PLoS One (2014) 0.77
A specific amyloid-beta protein assembly in the brain impairs memory. Nature (2006) 16.19
Adapting proteostasis for disease intervention. Science (2008) 13.22
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature (2004) 11.82
A beta oligomers - a decade of discovery. J Neurochem (2007) 8.88
Regulation of aging and age-related disease by DAF-16 and heat-shock factor. Science (2003) 8.43
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature (2007) 7.74
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell (1998) 7.13
Glutamine repeats and neurodegeneration. Annu Rev Neurosci (2000) 6.06
Opposing activities protect against age-onset proteotoxicity. Science (2006) 5.85
Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science (2006) 5.39
Modulation of neurodegeneration by molecular chaperones. Nat Rev Neurosci (2005) 5.00
Diseases of unstable repeat expansion: mechanisms and common principles. Nat Rev Genet (2005) 4.66
Artificial miRNAs mitigate shRNA-mediated toxicity in the brain: implications for the therapeutic development of RNAi. Proc Natl Acad Sci U S A (2008) 4.53
The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans. Proc Natl Acad Sci U S A (2002) 4.25
Global changes to the ubiquitin system in Huntington's disease. Nature (2007) 3.91
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature (2008) 3.68
Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation. Proc Natl Acad Sci U S A (2002) 3.56
ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell (2006) 3.39
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med (2005) 3.19
Accelerating amyloid-beta fibrillization reduces oligomer levels and functional deficits in Alzheimer disease mouse models. J Biol Chem (2007) 3.13
Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation. Proc Natl Acad Sci U S A (2004) 2.89
Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet (2007) 2.77
RNA toxicity is a component of ataxin-3 degeneration in Drosophila. Nature (2008) 2.74
Amyloid-like features of polyglutamine aggregates and their assembly kinetics. Biochemistry (2002) 2.59
The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions. Nat Cell Biol (2006) 2.58
Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. Nat Cell Biol (2006) 2.37
A toxic monomeric conformer of the polyglutamine protein. Nat Struct Mol Biol (2007) 2.33
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron (1999) 2.25
Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell (2004) 2.19
Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. Mol Cell (2006) 2.16
Deletion of the ubiquitin ligase CHIP leads to the accumulation, but not the aggregation, of both endogenous phospho- and caspase-3-cleaved tau species. J Neurosci (2006) 1.98
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. J Biol Chem (2005) 1.88
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A (2005) 1.87
The co-chaperone carboxyl terminus of Hsp70-interacting protein (CHIP) mediates alpha-synuclein degradation decisions between proteasomal and lysosomal pathways. J Biol Chem (2005) 1.85
Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila. PLoS Genet (2007) 1.66
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum Mol Genet (2007) 1.66
Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model. J Neurosci (2006) 1.65
The Hsp70 and TRiC/CCT chaperone systems cooperate in vivo to assemble the von Hippel-Lindau tumor suppressor complex. Mol Cell Biol (2003) 1.62
Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function. J Biol Chem (2003) 1.60
Molecular clearance of ataxin-3 is regulated by a mammalian E4. EMBO J (2004) 1.57
CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci (2005) 1.57
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J Biol Chem (2006) 1.55
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J Neurochem (2007) 1.54
Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration. Nat Neurosci (2007) 1.46
Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy. J Biol Chem (2006) 1.41
CAG-encoded polyglutamine length polymorphism in the human genome. BMC Genomics (2007) 1.36
CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. J Neurosci (2007) 1.34
Pathogenic and non-pathogenic polyglutamine tracts have similar structural properties: towards a length-dependent toxicity gradient. J Mol Biol (2007) 1.31
The structure of a polyQ-anti-polyQ complex reveals binding according to a linear lattice model. Nat Struct Mol Biol (2007) 1.29
Mechanisms of ataxin-3 misfolding and fibril formation: kinetic analysis of a disease-associated polyglutamine protein. J Mol Biol (2007) 1.29
Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease. J Neurosci (2005) 1.18
CHIP and HSPs interact with beta-APP in a proteasome-dependent manner and influence Abeta metabolism. Hum Mol Genet (2007) 1.16
The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils. PLoS One (2006) 1.15
Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum Mol Genet (2007) 1.13
The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes. J Neurosci (2007) 1.08
Side-chain interactions determine amyloid formation by model polyglutamine peptides in molecular dynamics simulations. Biophys J (2006) 1.02
E6-AP promotes misfolded polyglutamine proteins for proteasomal degradation and suppresses polyglutamine protein aggregation and toxicity. J Biol Chem (2008) 0.99
Co-chaperone CHIP promotes aggregation of ataxin-1. Mol Cell Neurosci (2006) 0.98
Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathol (2006) 0.91
siRNA-mediated gene silencing in vitro and in vivo. Nat Biotechnol (2002) 5.65
RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proc Natl Acad Sci U S A (2005) 4.79
RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia. Nat Med (2004) 4.63
Allele-specific silencing of dominant disease genes. Proc Natl Acad Sci U S A (2003) 3.00
CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron (2013) 2.94
RNA-mediated neurodegeneration in repeat expansion disorders. Ann Neurol (2010) 2.30
The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J Biol Chem (2008) 2.09
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
Spinocerebellar ataxias: an update. Curr Opin Neurol (2007) 1.48
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
Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3. J Neurosci (2011) 1.46
Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J (2009) 1.46
Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. J Biol Chem (2003) 1.44
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
Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3. J Biol Chem (2005) 1.34
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
Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet J Rare Dis (2013) 1.15
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
Cognitive decline and aging: the role of concussive and subconcussive impacts. Exerc Sport Sci Rev (2012) 1.11
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
Caring for Machado-Joseph disease: current understanding and how to help patients. Parkinsonism Relat Disord (2009) 0.88
Early alterations of autophagy in Huntington disease-like mice. Autophagy (2010) 0.87
Splice isoform-specific suppression of the Cav2.1 variant underlying spinocerebellar ataxia type 6. Neurobiol Dis (2011) 0.86
Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics. Curr Alzheimer Res (2012) 0.86
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
If it's not one thing, it's another. Nat Genet (2006) 0.76
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
Considering a career in neurology research? Heed these words. Ann Neurol (2013) 0.75