Published in Front Mol Neurosci on July 31, 2014
Parkinson's disease: animal models and dopaminergic cell vulnerability. Front Neuroanat (2014) 1.18
Neuronal aggregates: formation, clearance, and spreading. Dev Cell (2015) 1.09
Spermidine protects against α-synuclein neurotoxicity. Cell Cycle (2014) 1.02
Elusive roles for reactive astrocytes in neurodegenerative diseases. Front Cell Neurosci (2015) 0.95
An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice. Hum Mol Genet (2015) 0.86
It Is All about (U)biquitin: Role of Altered Ubiquitin-Proteasome System and UCHL1 in Alzheimer Disease. Oxid Med Cell Longev (2016) 0.82
The ubiquitin proteasomal system: a potential target for the management of Alzheimer's disease. J Cell Mol Med (2016) 0.81
Drosophila Neuronal Injury Follows a Temporal Sequence of Cellular Events Leading to Degeneration at the Neuromuscular Junction. J Exp Neurosci (2015) 0.81
Legal but lethal: functional protein aggregation at the verge of toxicity. Front Cell Neurosci (2015) 0.81
Protein misfolding specifies recruitment to cytoplasmic inclusion bodies. J Cell Biol (2016) 0.81
A dynamic interface between ubiquitylation and cAMP signaling. Front Pharmacol (2015) 0.80
De Novo Disruption of the Proteasome Regulatory Subunit PSMD12 Causes a Syndromic Neurodevelopmental Disorder. Am J Hum Genet (2017) 0.80
The life cycle of the 26S proteasome: from birth, through regulation and function, and onto its death. Cell Res (2016) 0.80
Proteostasis and RNA Binding Proteins in Synaptic Plasticity and in the Pathogenesis of Neuropsychiatric Disorders. Neural Plast (2016) 0.78
Chronic Toxoplasma gondii infection enhances β-amyloid phagocytosis and clearance by recruited monocytes. Acta Neuropathol Commun (2016) 0.78
Prion degradation pathways: Potential for therapeutic intervention. Mol Cell Neurosci (2015) 0.78
Sirtuins and proteolytic systems: implications for pathogenesis of synucleinopathies. Biomolecules (2015) 0.78
Perturbed proteostasis in autism spectrum disorders. J Neurochem (2016) 0.78
Accumulation of Ubiquitin and Sequestosome-1 Implicate Protein Damage in Diacetyl-Induced Cytotoxicity. Am J Pathol (2016) 0.78
Ubiquitin-dependent proteolysis in yeast cells expressing neurotoxic proteins. Front Mol Neurosci (2015) 0.77
Cross Talk of Proteostasis and Mitostasis in Cellular Homeodynamics, Ageing, and Disease. Oxid Med Cell Longev (2016) 0.77
Modulation of proteostasis by transcription factor NRF2 and impact in neurodegenerative diseases. Redox Biol (2017) 0.76
Ubiquitin-Synaptobrevin Fusion Protein Causes Degeneration of Presynaptic Motor Terminals in Mice. J Neurosci (2015) 0.76
Oligodendroglia and Myelin in Neurodegenerative Diseases: More Than Just Bystanders? Mol Neurobiol (2015) 0.76
Expression of immunoproteasome genes is regulated by cell-intrinsic and -extrinsic factors in human cancers. Sci Rep (2016) 0.76
A Central Role for Phosphorylated p38α in Linking Proteasome Inhibition-Induced Apoptosis and Autophagy. Mol Neurobiol (2016) 0.76
The Autophagoproteasome a Novel Cell Clearing Organelle in Baseline and Stimulated Conditions. Front Neuroanat (2016) 0.76
The Logic of the 26S Proteasome. Cell (2017) 0.76
Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly. Cell Death Dis (2017) 0.75
PolyQ 2.0: an improved version of PolyQ, a database of human polyglutamine proteins. Database (Oxford) (2016) 0.75
Protein misfolding in neurodegenerative diseases: implications and strategies. Transl Neurodegener (2017) 0.75
Expression profile of a Caenorhabditis elegans model of adult neuronal ceroid lipofuscinosis reveals down regulation of ubiquitin E3 ligase components. Sci Rep (2015) 0.75
Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders. Mol Neurobiol (2016) 0.75
Expression Atlas of the Deubiquitinating Enzymes in the Adult Mouse Retina, Their Evolutionary Diversification and Phenotypic Roles. PLoS One (2016) 0.75
Poly-Ubiquitinylation Profile in Down Syndrome Brain before and after the Development of Alzheimer Neuropathology. Antioxid Redox Signal (2016) 0.75
An assay for 26S proteasome activity based on fluorescence anisotropy measurements of dye-labeled protein substrates. Anal Biochem (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
The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity. Mol Neurobiol (2016) 0.75
Cigarette smoke induced autophagy-impairment regulates AMD pathogenesis mechanisms in ARPE-19 cells. PLoS One (2017) 0.75
Targeting the proteasome in epilepsy. Oncotarget (2017) 0.75
The ubiquitin system. Annu Rev Biochem (1998) 43.36
Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature (2006) 24.39
Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature (1998) 21.49
Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature (2006) 21.40
alpha-Synuclein locus triplication causes Parkinson's disease. Science (2003) 20.20
Mechanisms underlying ubiquitination. Annu Rev Biochem (2001) 18.87
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet (2004) 14.31
Drosophila pink1 is required for mitochondrial function and interacts genetically with parkin. Nature (2006) 11.91
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature (2004) 11.82
Mitochondrial dysfunction in Drosophila PINK1 mutants is complemented by parkin. Nature (2006) 11.81
Impairment of the ubiquitin-proteasome system by protein aggregation. Science (2001) 11.51
Aggresomes: a cellular response to misfolded proteins. J Cell Biol (1998) 11.24
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature (1991) 9.89
Trinucleotide repeat disorders. Annu Rev Neurosci (2007) 9.45
Aggresomes, inclusion bodies and protein aggregation. Trends Cell Biol (2000) 9.40
Dynamics and diversity in autophagy mechanisms: lessons from yeast. Nat Rev Mol Cell Biol (2009) 9.32
Recognition and processing of ubiquitin-protein conjugates by the proteasome. Annu Rev Biochem (2009) 8.83
Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron (2010) 7.87
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet (2004) 7.84
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature (2007) 7.74
The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress. Cell (2003) 7.71
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell (1997) 7.59
The ubiquitin code. Annu Rev Biochem (2012) 7.08
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell (2000) 6.45
Generation of destabilized green fluorescent protein as a transcription reporter. J Biol Chem (1998) 5.22
Autophagy inhibition compromises degradation of ubiquitin-proteasome pathway substrates. Mol Cell (2009) 5.18
Short-lived green fluorescent proteins for quantifying ubiquitin/proteasome-dependent proteolysis in living cells. Nat Biotechnol (2000) 5.14
Enhancement of proteasome activity by a small-molecule inhibitor of USP14. Nature (2010) 5.08
Ubiquitin is a component of paired helical filaments in Alzheimer's disease. Science (1987) 5.06
Misfolded proteins partition between two distinct quality control compartments. Nature (2008) 4.75
Reading protein modifications with interaction domains. Nat Rev Mol Cell Biol (2006) 4.55
Conformational disease. Lancet (1997) 4.53
The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg. Neuron (2003) 4.49
Yeast cells provide insight into alpha-synuclein biology and pathobiology. Science (2003) 4.45
Ornithine decarboxylase is degraded by the 26S proteasome without ubiquitination. Nature (1992) 4.43
Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron (2001) 4.43
Family-based association between Alzheimer's disease and variants in UBQLN1. N Engl J Med (2005) 4.34
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat Genet (1998) 4.27
Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera. J Cell Biol (1999) 4.06
Ubiquitin signals autophagic degradation of cytosolic proteins and peroxisomes. Proc Natl Acad Sci U S A (2008) 3.92
Global changes to the ubiquitin system in Huntington's disease. Nature (2007) 3.91
Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system. Nat Cell Biol (2012) 3.90
Selective autophagy: ubiquitin-mediated recognition and beyond. Nat Cell Biol (2010) 3.74
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease. J Cell Biol (2009) 3.50
Editing of ubiquitin conjugates by an isopeptidase in the 26S proteasome. Nature (1997) 3.47
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol Biol Cell (2001) 3.41
Delivery of ubiquitinated substrates to protein-unfolding machines. Nat Cell Biol (2005) 3.38
Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. Mol Cell (2005) 3.28
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med (2005) 3.19
Parkin-mediated K63-linked polyubiquitination targets misfolded DJ-1 to aggresomes via binding to HDAC6. J Cell Biol (2007) 3.13
Nanobodies: natural single-domain antibodies. Annu Rev Biochem (2013) 3.12
Expression of A53T mutant but not wild-type alpha-synuclein in PC12 cells induces alterations of the ubiquitin-dependent degradation system, loss of dopamine release, and autophagic cell death. J Neurosci (2001) 3.10
Polarised asymmetric inheritance of accumulated protein damage in higher eukaryotes. PLoS Biol (2006) 3.06
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD. Autophagy (2010) 2.99
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet (2000) 2.95
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins. Mol Cell (2004) 2.89
Active site mutants in the six regulatory particle ATPases reveal multiple roles for ATP in the proteasome. EMBO J (1998) 2.82
Monitoring activity and inhibition of 26S proteasomes with fluorogenic peptide substrates. Methods Enzymol (2005) 2.49
The pathways of mitophagy for quality control and clearance of mitochondria. Cell Death Differ (2012) 2.45
Lysine 63-linked ubiquitination promotes the formation and autophagic clearance of protein inclusions associated with neurodegenerative diseases. Hum Mol Genet (2007) 2.44
Impaired proteasome function in Alzheimer's disease. J Neurochem (2000) 2.44
Protacs: chimeric molecules that target proteins to the Skp1-Cullin-F box complex for ubiquitination and degradation. Proc Natl Acad Sci U S A (2001) 2.39
Repeat expansion disease: progress and puzzles in disease pathogenesis. Nat Rev Genet (2010) 2.36
Ubiquitin accumulation in autophagy-deficient mice is dependent on the Nrf2-mediated stress response pathway: a potential role for protein aggregation in autophagic substrate selection. J Cell Biol (2010) 2.32
Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice. J Neurosci (2004) 2.29
Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Hum Mol Genet (2001) 2.29
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Neuron (2009) 2.26
A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system. J Biol Chem (2002) 2.26
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice. Neuron (1999) 2.25
Inefficient degradation of truncated polyglutamine proteins by the proteasome. EMBO J (2004) 2.24
Transcription factor Nrf1 mediates the proteasome recovery pathway after proteasome inhibition in mammalian cells. Mol Cell (2010) 2.24
The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks. Nat Struct Mol Biol (2011) 2.23
Polyglutamine protein aggregates are dynamic. Nat Cell Biol (2002) 2.21
Antioxidants enhance mammalian proteasome expression through the Keap1-Nrf2 signaling pathway. Mol Cell Biol (2003) 2.19
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Hum Mol Genet (2001) 2.16
The ubiquitin-selective segregase VCP/p97 orchestrates the response to DNA double-strand breaks. Nat Cell Biol (2011) 2.15
The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum Mol Genet (2003) 2.13
Parkin suppresses dopaminergic neuron-selective neurotoxicity induced by Pael-R in Drosophila. Neuron (2003) 2.12
RPN-6 determines C. elegans longevity under proteotoxic stress conditions. Nature (2012) 2.11
The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. J Biol Chem (2008) 2.09
Cdc48: a power machine in protein degradation. Trends Biochem Sci (2011) 2.08
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation. Hum Mol Genet (2005) 2.01
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3. J Cell Biol (2006) 2.00
A transgenic mouse model of the ubiquitin/proteasome system. Nat Biotechnol (2003) 1.95
Generalized brain and skin proteasome inhibition in Huntington's disease. Ann Neurol (2004) 1.92
Aggregated and monomeric alpha-synuclein bind to the S6' proteasomal protein and inhibit proteasomal function. J Biol Chem (2003) 1.91
Altered proteasomal function in sporadic Parkinson's disease. Exp Neurol (2003) 1.91
Endoplasmic reticulum stress compromises the ubiquitin-proteasome system. Hum Mol Genet (2005) 1.90
Peptide diffusion, protection, and degradation in nuclear and cytoplasmic compartments before antigen presentation by MHC class I. Immunity (2003) 1.89
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function. Cell (2013) 1.88
Degradation of alpha-synuclein by proteasome. J Biol Chem (1999) 1.87
Harnessing the ubiquitination machinery to target the degradation of specific cellular proteins. Mol Cell (2000) 1.86
Ataxin-7 is a subunit of GCN5 histone acetyltransferase-containing complexes. Hum Mol Genet (2004) 1.85
Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol Cell (2005) 1.84
Neuronal induction of the immunoproteasome in Huntington's disease. J Neurosci (2003) 1.83
Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity. Genes Dev (2008) 1.81
Increased proteasome activity in human embryonic stem cells is regulated by PSMD11. Nature (2012) 1.75
Beta-amyloid accumulation impairs multivesicular body sorting by inhibiting the ubiquitin-proteasome system. J Neurosci (2006) 1.75
Non-canonical ubiquitin-based signals for proteasomal degradation. J Cell Sci (2012) 1.75