Published in Curr Opin Genet Dev on June 01, 2003
Yeast cells provide insight into alpha-synuclein biology and pathobiology. Science (2003) 4.45
Protein carbonylation, cellular dysfunction, and disease progression. J Cell Mol Med (2006) 2.59
Folding versus aggregation: polypeptide conformations on competing pathways. Arch Biochem Biophys (2007) 1.97
Genome wide analysis of common and specific stress responses in adult drosophila melanogaster. BMC Genomics (2004) 1.82
A mouse forward genetics screen identifies LISTERIN as an E3 ubiquitin ligase involved in neurodegeneration. Proc Natl Acad Sci U S A (2009) 1.74
Signaling, polyubiquitination, trafficking, and inclusions: sequestosome 1/p62's role in neurodegenerative disease. J Biomed Biotechnol (2006) 1.44
Toward understanding Machado-Joseph disease. Prog Neurobiol (2011) 1.42
Neural-specific deletion of FIP200 leads to cerebellar degeneration caused by increased neuronal death and axon degeneration. J Biol Chem (2009) 1.38
In vitro analysis of Hrd1p-mediated retrotranslocation of its multispanning membrane substrate 3-hydroxy-3-methylglutaryl (HMG)-CoA reductase. J Biol Chem (2009) 1.31
Modulation of polyglutamine conformations and dimer formation by the N-terminus of huntingtin. J Mol Biol (2009) 1.30
The ubiquitin-proteasome system and the autophagic-lysosomal system in Alzheimer disease. Cold Spring Harb Perspect Med (2012) 1.27
Protein folding: then and now. Arch Biochem Biophys (2007) 1.18
Assessing the contribution of heterogeneous distributions of oligomers to aggregation mechanisms of polyglutamine peptides. Biophys Chem (2011) 1.13
Alpha-synuclein targets the plasma membrane via the secretory pathway and induces toxicity in yeast. Genetics (2005) 1.09
Molecular chaperones as rational drug targets for Parkinson's disease therapeutics. CNS Neurol Disord Drug Targets (2010) 1.04
Sodium 4-phenylbutyrate ameliorates the effects of cataract-causing mutant gammaD-crystallin in cultured cells. Mol Vis (2010) 1.02
Slow degradation and aggregation in vitro of mutant GABAA receptor gamma2(Q351X) subunits associated with epilepsy. J Neurosci (2010) 0.99
Trafficking-deficient mutant GABRG2 subunit amount may modify epilepsy phenotype. Ann Neurol (2013) 0.95
The human epilepsy mutation GABRG2(Q390X) causes chronic subunit accumulation and neurodegeneration. Nat Neurosci (2015) 0.95
Mitochondrial dysfunction precedes neurodegeneration in mahogunin (Mgrn1) mutant mice. Neurobiol Aging (2007) 0.94
Clearance and phosphorylation of alpha-synuclein are inhibited in methionine sulfoxide reductase a null yeast cells. J Mol Neurosci (2009) 0.93
Chemical chaperone and inhibitor discovery: potential treatments for protein conformational diseases. Perspect Medicin Chem (2007) 0.90
C-terminus of heat shock cognate 70 interacting protein increases following stroke and impairs survival against acute oxidative stress. Antioxid Redox Signal (2010) 0.89
Dealing with misfolded proteins: examining the neuroprotective role of molecular chaperones in neurodegeneration. Molecules (2010) 0.86
The ubiquitin-proteasome system in spongiform degenerative disorders. Biochim Biophys Acta (2008) 0.86
Are heat shock proteins therapeutic target for Parkinson's disease? Int J Biol Sci (2006) 0.86
The therapeutic importance of understanding mechanisms of neuronal cell death in neurodegenerative disease. Mol Neurodegener (2009) 0.84
Decreased Phosphorylation and Increased Methionine Oxidation of α-Synuclein in the Methionine Sulfoxide Reductase A Knockout Mouse. J Amino Acids (2011) 0.82
System-wide analysis reveals intrinsically disordered proteins are prone to ubiquitylation after misfolding stress. Mol Cell Proteomics (2013) 0.82
Compromised mitochondrial complex II in models of Machado-Joseph disease. Biochim Biophys Acta (2011) 0.82
Ovarian steroids regulate gene expression related to DNA repair and neurodegenerative diseases in serotonin neurons of macaques. Mol Psychiatry (2015) 0.80
Reductions of the components of the calreticulin/calnexin quality-control system by proteasome inhibitors and their relevance in a rodent model of Parkinson's disease. J Neurosci Res (2014) 0.79
Hydrodynamic size-based separation and characterization of protein aggregates from total cell lysates. Nat Protoc (2014) 0.78
Mahogunin ring finger-1 (MGRN1) suppresses chaperone-associated misfolded protein aggregation and toxicity. Sci Rep (2013) 0.77
Role of Carbonyl Modifications on Aging-Associated Protein Aggregation. Sci Rep (2016) 0.76
Tau interactome mapping based identification of Otub1 as Tau deubiquitinase involved in accumulation of pathological Tau forms in vitro and in vivo. Acta Neuropathol (2017) 0.75
Tumor Necrosis Factor-Associated Protein 1 (TRAP1) is Released from the Mitochondria Following 6-hydroxydopamine Treatment. Exp Neurobiol (2014) 0.75
Motor Dysfunctions and Neuropathology in Mouse Models of Spinocerebellar Ataxia Type 2: A Comprehensive Review. Front Neurosci (2016) 0.75
Phosphorylation of the 19S regulatory particle ATPase subunit, Rpt6, modifies susceptibility to proteotoxic stress and protein aggregation. PLoS One (2017) 0.75
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
Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci (2008) 2.63
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
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
Spinocerebellar ataxias: an update. Curr Opin Neurol (2007) 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
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
Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice. Mol Ther (2013) 0.94
Analysis of the tau-associated proteome reveals that exchange of Hsp70 for Hsp90 is involved in tau degradation. ACS Chem Biol (2012) 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
New hope for therapy in neurodegenerative diseases. Cell Res (2013) 0.75
Drug discovery: Kill the messenger where it lives. Nature (2012) 0.75
Dangerous liaisons: polyglutamine meets HMGB. Nat Cell Biol (2007) 0.75
Considering a career in neurology research? Heed these words. Ann Neurol (2013) 0.75