Standout Papers

Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating ... 1999 2026 2008 2017 404
  1. Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice (1999)
    Christopher J. Cummings, Eyal Reinstein et al. Neuron

Citation Impact

Citing Papers

Neurotoxic protein expression reveals connections between the circadian clock and mating behavior in Drosophila
2006 StandoutNobel
Impairment of starvation-induced and constitutive autophagy in Atg7 -deficient mice
2005 StandoutNobel
Parkinson-like syndrome induced by continuous MPTP infusion: Convergent roles of the ubiquitin-proteasome system and α-synuclein
2005 StandoutNobel
Chaperone Suppression of α-Synuclein Toxicity in a Drosophila Model for Parkinson's Disease
2002 StandoutScience
No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis
2002
Parkin Facilitates the Elimination of Expanded Polyglutamine Proteins and Leads to Preservation of Proteasome Function
2003
Protein misfolding in Alzheimer’s and Parkinson’s disease: genetics and molecular mechanisms
2002
The NEDD8 Pathway Is Essential for SCFβ-TrCP-mediated Ubiquitination and Processing of the NF-κB Precursor p105
2002 StandoutNobel
Apoptosis in the nervous system
2000 StandoutNature
The Ubiquitin Proteasome System in Neurodegenerative Diseases
2003 StandoutNobel
Are Huntington’s and polyglutamine-based ataxias proteasome storage diseases?
2003
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
2002 StandoutNature
Determinants of Nuclear and Cytoplasmic Ubiquitin-mediated Degradation of MyoD
2003 StandoutNobel
Regulation of V2 Vasopressin Receptor Degradation by Agonist-promoted Ubiquitination
2003 StandoutNobel
Ubiquitin-Proteasome-mediated Degradation of Id1 Is Modulated by MyoD
2004 StandoutNobel
CD22 blockade restores homeostatic microglial phagocytosis in ageing brains
2019 StandoutNatureNobel
Molecular genetics: Unmasking polyglutamine triggers in neurodegenerative disease
2000
Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid β-peptide
2007 Standout
Proteolytic stress: A unifying concept for the etiopathogenesis of Parkinson's disease
2003
The role of the ubiquitin-proteasomal pathway in Parkinson's disease and other neurodegenerative disorders
2001
Intranuclear Inclusions and the Ubiquitin–Proteasome Pathway
1999
Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation
2005
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
2004 StandoutNature
Shared Principles in NF-κB Signaling
2008 Standout
Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex
2001
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release
2001
Autophagy fights disease through cellular self-digestion
2008 StandoutNature
Proteasome inhibition by paired helical filament‐tau in brains of patients with Alzheimer's disease
2003
Parkin Suppresses Dopaminergic Neuron-Selective Neurotoxicity Induced by Pael-R in Drosophila
2003
The Presynaptic Active Zone
2012 StandoutNobel
Autophagy: Renovation of Cells and Tissues
2011 Standout
Inefficient degradation of truncated polyglutamine proteins by the proteasome
2004
Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice
2006 StandoutNature
The Histone Chaperone Spt6 Is Required for Activation-induced Cytidine Deaminase Target Determination through H3K4me3 Regulation
2012 StandoutNobel
Suppressor mutations in Mecp2 -null mice implicate the DNA damage response in Rett syndrome pathology
2020
Protein degradation and protection against misfolded or damaged proteins
2003 StandoutNature
Proteasomal Inhibition-Induced Inclusion Formation and Death in Cortical Neurons Require Transcription and Ubiquitination
2002
Processing of p105 Is Inhibited by Docking of p50 Active Subunits to the Ankyrin Repeat Domain, and Inhibition Is Alleviated by Signaling via the Carboxyl-terminal Phosphorylation/ Ubiquitin-Ligase Binding Domain
2001 StandoutNobel
A transgenic mouse model of the ubiquitin/proteasome system
2003
Protein folding and misfolding
2003 StandoutNature
Mitochondrial Localization of Mutant Superoxide Dismutase 1 Triggers Caspase-dependent Cell Death in a Cellular Model of Familial Amyotrophic Lateral Sclerosis
2002
Molecular chaperones in protein folding and proteostasis
2011 StandoutNature
Tracking and Elucidating Alphavirus-Host Protein Interactions
2006 StandoutNobel
α-Synuclein Cooperates with CSPα in Preventing Neurodegeneration
2005 StandoutNobel
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
2001
Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein
2003
From endoplasmic-reticulum stress to the inflammatory response
2008 StandoutNature
Is there a cause-and-effect relationship between α-synuclein fibrillization and Parkinson’s disease?
2000
Parkin ubiquitinates the α-synuclein–interacting protein, synphilin-1: implications for Lewy-body formation in Parkinson disease
2001
SCA7 Knockin Mice Model Human SCA7 and Reveal Gradual Accumulation of Mutant Ataxin-7 in Neurons and Abnormalities in Short-Term Plasticity
2003
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins
2002
Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components
2002
Proteasomal inhibition leads to formation of ubiquitin/α‐synuclein‐immunoreactive inclusions in PC12 cells
2001
Identification of genes that modify ataxin-1-induced neurodegeneration
2000 Nature
Alzheimer's Disease
2010 Standout
The ubiquitin system: pathogenesis of human diseases and drug targeting
2004 StandoutNobel
Loss of autophagy in the central nervous system causes neurodegeneration in mice
2006 StandoutNature
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases
2006 StandoutNature
Repertoires of Autophagy in the Pathogenesis of Ocular Diseases
2015 Standout
Reversing Neurodegeneration:A Promise Unfolds
2000
Calcium Channel Regulation and Presynaptic Plasticity
2008
Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase
2000 Standout
The Tumor Suppressor Protein p16 and the Human Papillomavirus Oncoprotein-58 E7 Are Naturally Occurring Lysine-less Proteins That Are Degraded by the Ubiquitin System
2004 StandoutNobel
Cellular Defenses against Unfolded Proteins
2001
From fruit fly to bedside
2003
Cellular Toxicity of Polyglutamine Expansion Proteins
2004
Parkinson's Disease
2003 Standout
Nonimmunoglobulin target loci of activation-induced cytidine deaminase (AID) share unique features with immunoglobulin genes
2012 StandoutNobel
Decrease in topoisomerase I is responsible for activation-induced cytidine deaminase (AID)-dependent somatic hypermutation
2011 StandoutNobel
Dynamic interactions between 14-3-3 proteins and phosphoproteins regulate diverse cellular processes
2004
The role of the ubiquitin-proteasomal pathway in Parkinson's disease and other neurodegenerative disorders
2001
Topoisomerase 1 and Single-Strand Break Repair Modulate Transcription-Induced CAG Repeat Contraction in Human Cells
2011
Molecular Chaperones in the Cytosol: from Nascent Chain to Folded Protein
2002 StandoutScience
Small molecule probes to quantify the functional fraction of a specific protein in a cell with minimal folding equilibrium shifts
2014 StandoutNobel
Tau Suppression in a Neurodegenerative Mouse Model Improves Memory Function
2005 StandoutScience
Proteasomal-Dependent Aggregate Reversal and Absence of Cell Death in a Conditional Mouse Model of Huntington's Disease
2001
Failure of the ubiquitin–proteasome system in Parkinson's disease
2001
Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity
2003
Ubiquitination of a New Form of α-Synuclein by Parkin from Human Brain: Implications for Parkinson's Disease
2001 Science
HdmX stimulates Hdm2-mediated ubiquitination and degradation of p53
2003 StandoutNobel
Impaired Degradation of Mutant α-Synuclein by Chaperone-Mediated Autophagy
2004 StandoutScience
Recent advances in the genetics and pathogenesis of Parkinson disease
2002
The Ubiquitin-Proteasome Proteolytic Pathway: Destruction for the Sake of Construction
2002 StandoutNobel
Mutations in the DJ-1 Gene Associated with Autosomal Recessive Early-Onset Parkinsonism
2003 StandoutScience
Impairment of the Ubiquitin-Proteasome System by Protein Aggregation
2001 StandoutScience
Autophagy in Health and Disease: A Double-Edged Sword
2004 StandoutScience

Works of Yaling Sun being referenced

Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant Ca V 2.1 channels
2008
Balanced X chromosome inactivation patterns in the Rett syndrome brain
2002
A Long CAG Repeat in the Mouse Sca1 Locus Replicates SCA1 Features and Reveals the Impact of Protein Solubility on Selective Neurodegeneration
2002
Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice
1999 StandoutNobel
Rankless by CCL
2026