Published in Hum Mol Genet on October 15, 2002
Cellular strategies for controlling protein aggregation. Nat Rev Mol Cell Biol (2010) 3.54
Control of autophagy as a therapy for neurodegenerative disease. Nat Rev Neurol (2011) 2.38
Autophagy and p62 in cardiac proteinopathy. Circ Res (2011) 2.24
Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases. Proc Natl Acad Sci U S A (2006) 1.73
Aggresome formation and neurodegenerative diseases: therapeutic implications. Curr Med Chem (2008) 1.68
A cellular system that degrades misfolded proteins and protects against neurodegeneration. Mol Cell (2014) 1.63
Selective degradation of aggregate-prone CryAB mutants by HSPB1 is mediated by ubiquitin-proteasome pathways. J Mol Cell Cardiol (2010) 1.54
Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice. J Cell Biol (2008) 1.51
Autophagy in neurodegeneration and development. Biochim Biophys Acta (2008) 1.38
Protein misfolding disorders and macroautophagy. Curr Opin Cell Biol (2010) 1.36
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
Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease. J Biol Chem (2008) 1.25
Sequence- and species-dependence of proteasomal processivity. ACS Chem Biol (2012) 1.18
The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution. Front Mol Neurosci (2014) 1.10
Neuronal aggregates: formation, clearance, and spreading. Dev Cell (2015) 1.09
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species. J Cell Sci (2014) 1.06
Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress. Mol Cell Biol (2009) 1.05
Ubiquitin crosstalk connecting cellular processes. Cell Div (2006) 1.04
Puromycin-sensitive aminopeptidase is the major peptidase responsible for digesting polyglutamine sequences released by proteasomes during protein degradation. EMBO J (2007) 1.04
Dendritic spine loss and neurodegeneration is rescued by Rab11 in models of Huntington's disease. Cell Death Differ (2010) 1.04
Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias. Cerebellum (2014) 1.03
A critical evaluation of the ubiquitin-proteasome system in Parkinson's disease. Biochim Biophys Acta (2009) 1.02
Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins. Prog Neurobiol (2011) 1.00
The role of ALFY in selective autophagy. Cell Death Differ (2012) 1.00
The ubiquitin-proteasome pathway in Huntington's disease. ScientificWorldJournal (2008) 0.99
Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment. Proc Natl Acad Sci U S A (2009) 0.98
The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS One (2009) 0.97
Age at onset in Huntington's disease is modified by the autophagy pathway: implication of the V471A polymorphism in Atg7. Hum Genet (2010) 0.94
Puromycin-sensitive aminopeptidase protects against aggregation-prone proteins via autophagy. Hum Mol Genet (2010) 0.94
The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias. BMC Biochem (2007) 0.93
Bifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments. PLoS One (2011) 0.93
Enhancing protein disaggregation restores proteasome activity in aged cells. Aging (Albany NY) (2013) 0.92
Interplay between the ubiquitin-proteasome system and autophagy in proteinopathies. Int J Physiol Pathophysiol Pharmacol (2009) 0.92
Substrate recognition in nuclear protein quality control degradation is governed by exposed hydrophobicity that correlates with aggregation and insolubility. J Biol Chem (2013) 0.91
Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes. J Biol Chem (2013) 0.89
Aging is not associated with proteasome impairment in UPS reporter mice. PLoS One (2009) 0.88
The proteasome is an integral part of solar ultraviolet a radiation-induced gene expression. J Biol Chem (2009) 0.87
Intrabodies as neuroprotective therapeutics. Neurotherapeutics (2013) 0.87
Huntington's Disease and Striatal Signaling. Front Neuroanat (2011) 0.86
The Ubiquitin-Proteasome System in Huntington's Disease: Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue? Biochem Res Int (2012) 0.83
Rho Kinase Inhibition as a Therapeutic for Progressive Supranuclear Palsy and Corticobasal Degeneration. J Neurosci (2016) 0.83
Lithium and autophagy. ACS Chem Neurosci (2014) 0.82
The role of autophagy in Parkinson's disease: rotenone-based modeling. Behav Brain Funct (2013) 0.82
From pathways to targets: understanding the mechanisms behind polyglutamine disease. Biomed Res Int (2014) 0.81
The requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubility. J Cell Sci (2014) 0.81
Protein misfolding specifies recruitment to cytoplasmic inclusion bodies. J Cell Biol (2016) 0.81
Parkinson's disease and autophagy. Parkinsons Dis (2012) 0.81
Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies. Mol Neurobiol (2013) 0.81
Evidence for prion-like mechanisms in several neurodegenerative diseases: potential implications for immunotherapy. Clin Dev Immunol (2013) 0.81
Promoting autophagic clearance: viable therapeutic targets in Alzheimer's disease. Neurotherapeutics (2015) 0.80
Possible role of the transglutaminases in the pathogenesis of Alzheimer's disease and other neurodegenerative diseases. Int J Alzheimers Dis (2011) 0.80
Cathepsins L and Z are critical in degrading polyglutamine-containing proteins within lysosomes. J Biol Chem (2012) 0.80
Pathogenic polyglutamine expansion length correlates with polarity of the flanking sequences. Mol Neurodegener (2014) 0.79
BimEL as a possible molecular link between proteasome dysfunction and cell death induced by mutant huntingtin. Eur J Neurosci (2010) 0.79
Inducible HSP70 is critical in preventing the aggregation and enhancing the processing of PMP22. ASN Neuro (2015) 0.79
Structural basis for mutation-induced destabilization of profilin 1 in ALS. Proc Natl Acad Sci U S A (2015) 0.79
The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients. PLoS One (2013) 0.79
MAP1B Interaction with the FW Domain of the Autophagic Receptor Nbr1 Facilitates Its Association to the Microtubule Network. Int J Cell Biol (2012) 0.79
p62 plays a protective role in the autophagic degradation of polyglutamine protein oligomers in polyglutamine disease model flies. J Biol Chem (2014) 0.78
Differential degradation of full-length and cleaved ataxin-7 fragments in a novel stable inducible SCA7 model. J Mol Neurosci (2012) 0.77
Degradation of oxidized proteins by the proteasome: Distinguishing between the 20S, 26S, and immunoproteasome proteolytic pathways. Mol Aspects Med (2016) 0.77
The E3 ubiquitin ligase seven in absentia homolog 1 may be a potential new therapeutic target for Parkinson's disease. Neural Regen Res (2015) 0.76
NLRP3 Localizes to the Tubular Epithelium in Human Kidney and Correlates With Outcome in IgA Nephropathy. Sci Rep (2016) 0.76
Neurodegeneration caused by polyglutamine expansion is regulated by P-glycoprotein in Drosophila melanogaster. Genetics (2013) 0.76
The Ginkgo biloba Extract EGb 761 Modulates Proteasome Activity and Polyglutamine Protein Aggregation. Evid Based Complement Alternat Med (2014) 0.75
The Impact of Paeoniflorin on α-Synuclein Degradation Pathway. Evid Based Complement Alternat Med (2015) 0.75
Ubiquitin versus misfolding: The minimal requirements for inclusion body formation. J Cell Biol (2016) 0.75
Aging and SKN-1-dependent Loss of 20S Proteasome Adaptation to Oxidative Stress in C. elegans. J Gerontol A Biol Sci Med Sci (2016) 0.75
Distinct Prion Domain Sequences Ensure Efficient Amyloid Propagation by Promoting Chaperone Binding or Processing In Vivo. PLoS Genet (2016) 0.75
PIN1 Modulates Huntingtin Levels and Aggregate Accumulation: An In vitro Model. Front Cell Neurosci (2017) 0.75
LC3A Silencing Hinders Aggresome Vimentin Cage Clearance in Primary Choroid Plexus Carcinoma. Sci Rep (2017) 0.75
Inhibition of proteasome deubiquitinating activity as a new cancer therapy. Nat Med (2011) 4.09
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD. Autophagy (2010) 2.99
Heterochromatin protein 1 is recruited to various types of DNA damage. J Cell Biol (2009) 2.26
The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks. Nat Struct Mol Biol (2011) 2.23
DNA damage triggers nucleotide excision repair-dependent monoubiquitylation of histone H2A. Genes Dev (2006) 2.22
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
A transgenic mouse model of the ubiquitin/proteasome system. Nat Biotechnol (2003) 1.95
Endoplasmic reticulum stress compromises the ubiquitin-proteasome system. Hum Mol Genet (2005) 1.90
Viral immune modulators perturb the human molecular network by common and unique strategies. Nature (2012) 1.86
Nucleotide excision repair-induced H2A ubiquitination is dependent on MDC1 and RNF8 and reveals a universal DNA damage response. J Cell Biol (2009) 1.77
The translation initiation factor 3f (eIF3f) exhibits a deubiquitinase activity regulating Notch activation. PLoS Biol (2010) 1.77
Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Hum Mol Genet (2005) 1.73
Activity-based ubiquitin-specific protease (USP) profiling of virus-infected and malignant human cells. Proc Natl Acad Sci U S A (2004) 1.59
The Epstein-Barr virus nuclear antigen-1 promotes genomic instability via induction of reactive oxygen species. Proc Natl Acad Sci U S A (2009) 1.57
A fluorescent broad-spectrum proteasome inhibitor for labeling proteasomes in vitro and in vivo. Chem Biol (2006) 1.54
A conserved unfoldase activity for the p97 AAA-ATPase in proteasomal degradation. J Mol Biol (2009) 1.54
The ER-resident ubiquitin-specific protease 19 participates in the UPR and rescues ERAD substrates. EMBO Rep (2009) 1.42
Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation. J Cell Biol (2002) 1.42
DDB2 promotes chromatin decondensation at UV-induced DNA damage. J Cell Biol (2012) 1.34
The UBA2 domain functions as an intrinsic stabilization signal that protects Rad23 from proteasomal degradation. Mol Cell (2005) 1.30
The Haemophilus ducreyi cytolethal distending toxin activates sensors of DNA damage and repair complexes in proliferating and non-proliferating cells. Cell Microbiol (2002) 1.26
Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis. Hum Mol Genet (2008) 1.24
A new non-catalytic role for ubiquitin ligase RNF8 in unfolding higher-order chromatin structure. EMBO J (2012) 1.24
Regulation of expression of Bcl-2 protein family member Bim by T cell receptor triggering. Proc Natl Acad Sci U S A (2004) 1.20
Activity profiling of deubiquitinating enzymes in cervical carcinoma biopsies and cell lines. Mol Carcinog (2006) 1.17
A deneddylase encoded by Epstein-Barr virus promotes viral DNA replication by regulating the activity of cullin-RING ligases. Nat Cell Biol (2010) 1.17
Dose-dependent inhibition of proteasome activity by a mutant ubiquitin associated with neurodegenerative disease. J Cell Sci (2007) 1.11
The Us3 protein kinase of herpes simplex virus 1 blocks apoptosis and induces phosporylation of the Bcl-2 family member Bad. Exp Cell Res (2003) 1.10
Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation. J Neurosci (2010) 1.10
Functional inactivation of EBV-specific T-lymphocytes in nasopharyngeal carcinoma: implications for tumor immunotherapy. PLoS One (2007) 1.07
Epstein-Barr virus: induction and control of cell transformation. J Cell Physiol (2003) 1.07
The ubiquitin specific protease 4 (USP4) is a new player in the Wnt signalling pathway. J Cell Mol Med (2009) 1.06
Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress. Mol Cell Biol (2009) 1.05
Ubiquitin crosstalk connecting cellular processes. Cell Div (2006) 1.04
Minimal length requirement for proteasomal degradation of ubiquitin-dependent substrates. FASEB J (2008) 1.03
Alzheimer's disease-associated ubiquilin-1 regulates presenilin-1 accumulation and aggresome formation. Traffic (2011) 1.02
Fluorescent probes for proteolysis: tools for drug discovery. Nat Rev Drug Discov (2004) 1.02
Stressing the ubiquitin-proteasome system. Cardiovasc Res (2009) 1.01
Ubiquitin-specific protease 19 (USP19) regulates hypoxia-inducible factor 1α (HIF-1α) during hypoxia. J Biol Chem (2011) 1.01
Capacity of Epstein-Barr virus to infect monocytes and inhibit their development into dendritic cells is affected by the cell type supporting virus replication. J Gen Virol (2004) 1.01
Epstein-barr virus encodes three bona fide ubiquitin-specific proteases. J Virol (2008) 1.01
MYC overexpression imposes a nonimmunogenic phenotype on Epstein-Barr virus-infected B cells. Proc Natl Acad Sci U S A (2002) 1.01
Chronic exposure to the cytolethal distending toxins of Gram-negative bacteria promotes genomic instability and altered DNA damage response. Cell Microbiol (2012) 1.00
A bacterial cytotoxin identifies the RhoA exchange factor Net1 as a key effector in the response to DNA damage. PLoS One (2008) 1.00
Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment. Proc Natl Acad Sci U S A (2009) 0.98
C-terminal UBA domains protect ubiquitin receptors by preventing initiation of protein degradation. Nat Commun (2011) 0.96
Monitoring of ubiquitin-dependent proteolysis with green fluorescent protein substrates. Methods Enzymol (2005) 0.95
Expression of immune-related molecules in primary EBV-positive Chinese nasopharyngeal carcinoma: associated with latent membrane protein 1 (LMP1) expression. Cancer Biol Ther (2007) 0.92
The hepatitis C virus core protein modulates T cell responses by inducing spontaneous and altering T-cell receptor-triggered Ca2+ oscillations. J Biol Chem (2003) 0.91
Functional p53 chimeras containing the Epstein-Barr virus Gly-Ala repeat are protected from Mdm2- and HPV-E6-induced proteolysis. Proc Natl Acad Sci U S A (2002) 0.91
Transcriptional profiling enables molecular classification of adrenocortical tumours. Eur J Endocrinol (2009) 0.91
The ubiquitin C-terminal hydrolase UCH-L1 regulates B-cell proliferation and integrin activation. J Cell Mol Med (2009) 0.90
Proteasome inhibition up-regulates p53 and apoptosis-inducing factor in chondrocytes causing severe growth retardation in mice. Cancer Res (2007) 0.90
Manipulation of immune responses by Epstein-Barr virus. Virus Res (2002) 0.89
Stabilization signals: a novel regulatory mechanism in the ubiquitin/proteasome system. FEBS Lett (2002) 0.89
Recognition of DNA damage by XPC coincides with disruption of the XPC-RAD23 complex. J Cell Biol (2012) 0.89
Transcription profiling of Epstein-Barr virus nuclear antigen (EBNA)-1 expressing cells suggests targeting of chromatin remodeling complexes. PLoS One (2010) 0.87
EBV and genomic instability--a new look at the role of the virus in the pathogenesis of Burkitt's lymphoma. Semin Cancer Biol (2009) 0.87
Mutant ubiquitin (UBB+1) associated with neurodegenerative disorders is hydrolyzed by ubiquitin C-terminal hydrolase L3 (UCH-L3). FEBS Lett (2011) 0.87
Caspase-1 promotes Epstein-Barr virus replication by targeting the large tegument protein deneddylase to the nucleus of productively infected cells. PLoS Pathog (2013) 0.86
Ubiquitin C-terminal hydrolase-L1 interacts with adhesion complexes and promotes cell migration, survival, and anchorage independent growth. FASEB J (2012) 0.86
Inhibition of ubiquitin/proteasome-dependent proteolysis in Saccharomyces cerevisiae by a Gly-Ala repeat. FEBS Lett (2003) 0.86
Hepatitis C virus core protein induces an anergic state characterized by decreased interleukin-2 production and perturbation of mitogen-activated protein kinase responses. J Virol (2005) 0.85
Proteasome inhibitors reconstitute the presentation of cytotoxic T-cell epitopes in Epstein-Barr virus-associated tumors. Int J Cancer (2002) 0.84
Bacterial genotoxin triggers FEN1-dependent RhoA activation, cytoskeleton remodeling and cell survival. J Cell Sci (2011) 0.84
The Epstein-Barr virus nuclear antigen-1 reprograms transcription by mimicry of high mobility group A proteins. Nucleic Acids Res (2013) 0.84
Illuminating the ubiquitin/proteasome system. Exp Cell Res (2010) 0.84
TPPII promotes genetic instability by allowing the escape from apoptosis of cells with activated mitotic checkpoints. Biochem Biophys Res Commun (2006) 0.83
Mitotic infidelity and centrosome duplication errors in cells overexpressing tripeptidyl-peptidase II. Cancer Res (2005) 0.83
Herpes virus deneddylases interrupt the cullin-RING ligase neddylation cycle by inhibiting the binding of CAND1. J Mol Cell Biol (2012) 0.82
Mutant p62/SQSTM1 UBA domains linked to Paget's disease of bone differ in their abilities to function as stabilization signals. FEBS Lett (2010) 0.82
Tetramer binding and secretion of interferon-gamma in response to antigenic stimulation are compatible with a range of affinities of MHC:TCR interaction and distinct programs of cytotoxic T-lymphocyte activation. Hum Immunol (2002) 0.81
Helicobacter pylori affects the cellular deubiquitinase USP7 and ubiquitin-regulated components TRAF6 and the tumour suppressor p53. Int J Med Microbiol (2010) 0.81
High avidity binding to DNA protects ubiquitylated substrates from proteasomal degradation. J Biol Chem (2011) 0.81
Fluorescent reporters for the ubiquitin-proteasome system. Essays Biochem (2005) 0.80
Regulation of lck degradation and refractory state in CD8+ cytotoxic T lymphocytes. Proc Natl Acad Sci U S A (2005) 0.80
Monitoring the ubiquitin/proteasome system in conformational diseases. Ageing Res Rev (2003) 0.80
The ubiquitin/proteasome system in Epstein-Barr virus latency and associated malignancies. Semin Cancer Biol (2003) 0.80
Involvement of ubiquilin-1 transcript variants in protein degradation and accumulation. Commun Integr Biol (2011) 0.80
Inhibition of ubiquitin-dependent proteolysis by a synthetic glycine-alanine repeat peptide that mimics an inhibitory viral sequence. FEBS Lett (2002) 0.80
Epstein-Barr virus encoded microRNAs target SUMO-regulated cellular functions. FEBS J (2014) 0.79
COPI-mediated retrograde transport is required for efficient gamma-secretase cleavage of the amyloid precursor protein. Biochem Biophys Res Commun (2006) 0.79
Pathways accessory to proteasomal proteolysis are less efficient in major histocompatibility complex class I antigen production. J Biol Chem (2002) 0.79
An N-terminal SIAH-interacting motif regulates the stability of the ubiquitin specific protease (USP)-19. Biochem Biophys Res Commun (2013) 0.77
The MAPK signaling cascade is a central hub in the regulation of cell cycle, apoptosis and cytoskeleton remodeling by tripeptidyl-peptidase II. Gene Regul Syst Bio (2008) 0.77
The Gly-Ala repeat modulates the interaction of Epstein-Barr virus nuclear antigen-1 with cellular chromatin. Biochem Biophys Res Commun (2013) 0.77
The ubiquitin specific protease-4 (USP4) interacts with the S9/Rpn6 subunit of the proteasome. Biochem Biophys Res Commun (2012) 0.77
Non-infectious fluorimetric assay for phenotyping of drug-resistant HIV proteinase mutants. J Clin Virol (2006) 0.77
Biogenesis and processing of the amyloid precursor protein in the early secretory pathway. Biochem Biophys Res Commun (2007) 0.77