Published in Mol Cell on April 27, 2007
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18
Sustained translational repression by eIF2α-P mediates prion neurodegeneration. Nature (2012) 3.54
Parkin-mediated K63-linked polyubiquitination targets misfolded DJ-1 to aggresomes via binding to HDAC6. J Cell Biol (2007) 3.13
Molecular mechanisms of proteasome assembly. Nat Rev Mol Cell Biol (2009) 2.82
Getting a grip on prions: oligomers, amyloids, and pathological membrane interactions. Annu Rev Biochem (2009) 2.80
The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases. EMBO J (2008) 2.43
Functional depletion of mahogunin by cytosolically exposed prion protein contributes to neurodegeneration. Cell (2009) 2.14
CHIP deficiency decreases longevity, with accelerated aging phenotypes accompanied by altered protein quality control. Mol Cell Biol (2008) 1.82
The ubiquitin proteasome system in neuropathology. Acta Neuropathol (2009) 1.65
Reduced translocation of nascent prion protein during ER stress contributes to neurodegeneration. Dev Cell (2008) 1.54
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathol (2014) 1.54
Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice. J Cell Biol (2008) 1.51
The concept of translocational regulation. J Cell Biol (2008) 1.50
Parkin-mediated K63-linked polyubiquitination: a signal for targeting misfolded proteins to the aggresome-autophagy pathway. Autophagy (2007) 1.50
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies. Exp Mol Med (2015) 1.29
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
Protein quality control and degradation in cardiomyocytes. J Mol Cell Cardiol (2008) 1.22
Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry. EMBO J (2011) 1.13
Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress. PLoS One (2010) 1.11
Proteasome functional insufficiency in cardiac pathogenesis. Am J Physiol Heart Circ Physiol (2011) 1.11
The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution. Front Mol Neurosci (2014) 1.10
Analysis of quality control substrates in distinct cellular compartments reveals a unique role for Rpn4p in tolerating misfolded membrane proteins. Mol Biol Cell (2008) 1.09
Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress. Mol Cell Biol (2009) 1.05
Signal sequence insufficiency contributes to neurodegeneration caused by transmembrane prion protein. J Cell Biol (2010) 1.04
Oligomers of mutant glial fibrillary acidic protein (GFAP) Inhibit the proteasome system in alexander disease astrocytes, and the small heat shock protein alphaB-crystallin reverses the inhibition. J Biol Chem (2010) 1.03
A critical evaluation of the ubiquitin-proteasome system in Parkinson's disease. Biochim Biophys Acta (2009) 1.02
Prion diseases: from protein to cell pathology. Am J Pathol (2008) 1.01
Methionine sulfoxides on prion protein Helix-3 switch on the alpha-fold destabilization required for conversion. PLoS One (2009) 1.01
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
Prion protein biosynthesis and its emerging role in neurodegeneration. Trends Biochem Sci (2009) 0.97
Role of protein misfolding and proteostasis deficiency in protein misfolding diseases and aging. Int J Cell Biol (2013) 0.97
Physiological and environmental control of yeast prions. FEMS Microbiol Rev (2013) 0.97
E2-25K/Hip-2 regulates caspase-12 in ER stress-mediated Abeta neurotoxicity. J Cell Biol (2008) 0.97
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
Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction. PLoS One (2011) 0.96
Changes in proteasome structure and function caused by HAMLET in tumor cells. PLoS One (2009) 0.95
Context dependent neuroprotective properties of prion protein (PrP). Prion (2009) 0.95
Proteasomal dysfunction and endoplasmic reticulum stress enhance trafficking of prion protein aggregates through the secretory pathway and increase accumulation of pathologic prion protein. J Biol Chem (2011) 0.94
Molecular pathology of human prion diseases. Int J Mol Sci (2009) 0.94
Alternative fates of newly formed PrPSc upon prion conversion on the plasma membrane. J Cell Sci (2013) 0.93
Activation of the macroautophagic system in scrapie-infected experimental animals and human genetic prion diseases. Autophagy (2012) 0.93
cAMP-induced phosphorylation of 26S proteasomes on Rpn6/PSMD11 enhances their activity and the degradation of misfolded proteins. Proc Natl Acad Sci U S A (2015) 0.93
The ubiquitin-proteasome system and cardiovascular disease. Prog Mol Biol Transl Sci (2012) 0.93
Interplay between the ubiquitin-proteasome system and autophagy in proteinopathies. Int J Physiol Pathophysiol Pharmacol (2009) 0.92
Type 2 diabetes as a protein misfolding disease. Trends Mol Med (2015) 0.91
Proteasome dysfunction triggers activation of SKN-1A/Nrf1 by the aspartic protease DDI-1. Elife (2016) 0.89
Aging is not associated with proteasome impairment in UPS reporter mice. PLoS One (2009) 0.88
Protein Quality Control by Molecular Chaperones in Neurodegeneration. Front Neurosci (2017) 0.87
Ubiquitin-specific protease 14 modulates degradation of cellular prion protein. Sci Rep (2015) 0.87
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders. Commun Integr Biol (2011) 0.87
Conformational properties of beta-PrP. J Biol Chem (2009) 0.87
Cytoplasmic prion protein induces forebrain neurotoxicity. Biochim Biophys Acta (2009) 0.86
The ubiquitin-proteasome system in spongiform degenerative disorders. Biochim Biophys Acta (2008) 0.86
Increased expression of p62/SQSTM1 in prion diseases and its association with pathogenic prion protein. Sci Rep (2014) 0.85
Regulation of the calpain and ubiquitin-proteasome systems in a canine model of muscular dystrophy. Muscle Nerve (2011) 0.83
Transcriptional changes in the brains of cattle orally infected with the bovine spongiform encephalopathy agent precede detection of infectivity. J Virol (2009) 0.83
Misfolded PrP and a novel mechanism of proteasome inhibition. Prion (2012) 0.83
Neuronal death induced by misfolded prion protein is due to NAD+ depletion and can be relieved in vitro and in vivo by NAD+ replenishment. Brain (2015) 0.82
Interactions of prion protein with intracellular proteins: so many partners and no consequences? Cell Mol Neurobiol (2009) 0.79
Activity-based imaging probes of the proteasome. Cell Biochem Biophys (2013) 0.79
Do amyloid oligomers act as traps for misfolded proteins? A hypothesis. Amyloid (2008) 0.79
Dendritic cell-mediated-immunization with xenogenic PrP and adenoviral vectors breaks tolerance and prolongs mice survival against experimental scrapie. PLoS One (2009) 0.78
N-terminal domain of prion protein directs its oligomeric association. J Biol Chem (2014) 0.78
Prion degradation pathways: Potential for therapeutic intervention. Mol Cell Neurosci (2015) 0.78
Proteomics analysis of amyloid and nonamyloid prion disease phenotypes reveals both common and divergent mechanisms of neuropathogenesis. J Proteome Res (2014) 0.77
Mechanistic insights into the cure of prion disease by novel antiprion compounds. J Virol (2007) 0.77
Prion-mediated neurodegeneration is associated with early impairment of the ubiquitin-proteasome system. Acta Neuropathol (2015) 0.77
Physical and Genetic Interactions Between Uls1 and the Slx5-Slx8 SUMO-Targeted Ubiquitin Ligase. G3 (Bethesda) (2013) 0.77
Cytosolic PrP can participate in prion-mediated toxicity. J Virol (2014) 0.77
Overexpression of PLK3 Mediates the Degradation of Abnormal Prion Proteins Dependent on Chaperone-Mediated Autophagy. Mol Neurobiol (2016) 0.76
Mechanisms of in vivo ribosome maintenance change in response to nutrient signals. Mol Cell Proteomics (2016) 0.75
Evaluating age-associated phenotypes in a mouse model of protein dyshomeostasis. Methods (2010) 0.75
Metabolism of minor isoforms of prion proteins: Cytosolic prion protein and transmembrane prion protein. Neural Regen Res (2013) 0.75
Prions--not your immunologist's pathogen. PLoS Pathog (2015) 0.75
Solvent microenvironments and copper binding alters the conformation and toxicity of a prion fragment. PLoS One (2013) 0.75
Disturbed vesicular trafficking of membrane proteins in prion disease. Prion (2013) 0.75
The heat shock response is modulated by and interferes with toxic effects of scrapie prion protein and amyloid β. J Biol Chem (2012) 0.75
Dual role of cellular prion protein in normal host and Alzheimer's disease. Proc Jpn Acad Ser B Phys Biol Sci (2017) 0.75
Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease. Nat Genet (2009) 15.15
Exome sequencing in sporadic autism spectrum disorders identifies severe de novo mutations. Nat Genet (2011) 11.94
TREM2 variants in Alzheimer's disease. N Engl J Med (2012) 11.35
Common variants at ABCA7, MS4A6A/MS4A4E, EPHA1, CD33 and CD2AP are associated with Alzheimer's disease. Nat Genet (2011) 9.23
A general model of prion strains and their pathogenicity. Science (2007) 6.94
Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol (2011) 4.98
Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science (2003) 4.57
Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet (2005) 4.21
Potent activity of carfilzomib, a novel, irreversible inhibitor of the ubiquitin-proteasome pathway, against preclinical models of multiple myeloma. Blood (2007) 3.67
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science (2003) 3.59
Chemistry-based functional proteomics reveals novel members of the deubiquitinating enzyme family. Chem Biol (2002) 3.43
A novel orally active proteasome inhibitor induces apoptosis in multiple myeloma cells with mechanisms distinct from Bortezomib. Cancer Cell (2005) 3.41
PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death. Mol Cell (2009) 3.17
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet (2006) 3.12
Design and use of conditional MHC class I ligands. Nat Med (2006) 3.01
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD. Autophagy (2010) 2.99
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J (2002) 2.98
Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science (2004) 2.82
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Loss of HAUSP-mediated deubiquitination contributes to DNA damage-induced destabilization of Hdmx and Hdm2. Mol Cell (2005) 2.76
Structure of the ubiquitin hydrolase UCH-L3 complexed with a suicide substrate. J Biol Chem (2004) 2.75
Prion propagation and toxicity in vivo occur in two distinct mechanistic phases. Nature (2011) 2.74
Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. Brain (2012) 2.65
Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60
Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron (2007) 2.58
Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of Alzheimer's disease. PLoS One (2010) 2.57
Crystal structure of the boronic acid-based proteasome inhibitor bortezomib in complex with the yeast 20S proteasome. Structure (2006) 2.56
Generation of peptide-MHC class I complexes through UV-mediated ligand exchange. Nat Protoc (2006) 2.47
Autoantibodies in sporadic Creutzfeldt-Jakob disease. JAMA Neurol (2013) 2.42
Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain (2003) 2.42
Selective induction of chemotherapy resistance of mammary tumors in a conditional mouse model for hereditary breast cancer. Proc Natl Acad Sci U S A (2007) 2.39
PINK1 is necessary for long term survival and mitochondrial function in human dopaminergic neurons. PLoS One (2008) 2.33
Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature (2003) 2.31
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
Heterochromatin protein 1 is recruited to various types of DNA damage. J Cell Biol (2009) 2.26
A systematic review of prion therapeutics in experimental models. Brain (2006) 2.25
Zinc-finger protein A20, a regulator of inflammation and cell survival, has de-ubiquitinating activity. Biochem J (2004) 2.23
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
A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain (2008) 2.13
Differential dependence of CD4+CD25+ regulatory and natural killer-like T cells on signals leading to NF-kappaB activation. Proc Natl Acad Sci U S A (2004) 2.09
Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. Am J Hum Genet (2013) 2.09
Stabilization of the transcription factor Foxp3 by the deubiquitinase USP7 increases Treg-cell-suppressive capacity. Immunity (2013) 2.05
OTU deubiquitinases reveal mechanisms of linkage specificity and enable ubiquitin chain restriction analysis. Cell (2013) 2.05
Ecology and behavior of first instar larval Lepidoptera. Annu Rev Entomol (2002) 1.99
Analysis of 2000 consecutive UK tonsillectomy specimens for disease-related prion protein. Lancet (2004) 1.96
Interaction between prion protein and toxic amyloid β assemblies can be therapeutically targeted at multiple sites. Nat Commun (2011) 1.96
A transgenic mouse model of the ubiquitin/proteasome system. Nat Biotechnol (2003) 1.95
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
Microglial activation in presymptomatic Huntington's disease gene carriers. Brain (2007) 1.93
Amyloid beta-protein dimers rapidly form stable synaptotoxic protofibrils. J Neurosci (2010) 1.92
Endoplasmic reticulum stress compromises the ubiquitin-proteasome system. Hum Mol Genet (2005) 1.90
Specific and covalent targeting of conjugating and deconjugating enzymes of ubiquitin-like proteins. Mol Cell Biol (2004) 1.86
Variant CJD in an individual heterozygous for PRNP codon 129. Lancet (2009) 1.84
Chemical synthesis of ubiquitin, ubiquitin-based probes, and diubiquitin. Angew Chem Int Ed Engl (2010) 1.83
Structural basis of substrate discrimination and integrin binding by autotaxin. Nat Struct Mol Biol (2011) 1.81
Boronic acid-based inhibitor of autotaxin reveals rapid turnover of LPA in the circulation. Proc Natl Acad Sci U S A (2010) 1.80
Activity probe for in vivo profiling of the specificity of proteasome inhibitor bortezomib. Nat Methods (2005) 1.78
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
Conditional MHC class I ligands and peptide exchange technology for the human MHC gene products HLA-A1, -A3, -A11, and -B7. Proc Natl Acad Sci U S A (2008) 1.77
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J (2008) 1.77
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
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
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins. Hum Mol Genet (2002) 1.73
Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc Natl Acad Sci U S A (2008) 1.71
A novel prion disease associated with diarrhea and autonomic neuropathy. N Engl J Med (2013) 1.69
Combinations of genetic mutations in the adult neural stem cell compartment determine brain tumour phenotypes. EMBO J (2009) 1.66
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res (2007) 1.59
The differential modulation of USP activity by internal regulatory domains, interactors and eight ubiquitin chain types. Chem Biol (2011) 1.58
Mechanism of USP7/HAUSP activation by its C-terminal ubiquitin-like domain and allosteric regulation by GMP-synthetase. Mol Cell (2011) 1.56
An ankyrin-repeat ubiquitin-binding domain determines TRABID's specificity for atypical ubiquitin chains. Nat Struct Mol Biol (2011) 1.55
CEP-18770: A novel, orally active proteasome inhibitor with a tumor-selective pharmacologic profile competitive with bortezomib. Blood (2007) 1.54
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
Comparing the hybrid fluorescent-radioactive tracer indocyanine green-99mTc-nanocolloid with 99mTc-nanocolloid for sentinel node identification: a validation study using lymphoscintigraphy and SPECT/CT. J Nucl Med (2012) 1.53
A novel protective prion protein variant that colocalizes with kuru exposure. N Engl J Med (2009) 1.53
Gene expression in Huntington's disease skeletal muscle: a potential biomarker. Hum Mol Genet (2005) 1.51
White matter connections reflect changes in voluntary-guided saccades in pre-symptomatic Huntington's disease. Brain (2007) 1.47
Update on human prion disease. Biochim Biophys Acta (2007) 1.46
Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY. PLoS Curr (2010) 1.45
Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors. Brain (2011) 1.45
Molecular and clinical classification of human prion disease. Br Med Bull (2003) 1.45
The deubiquitinating enzyme UCH-L3 regulates the apical membrane recycling of the epithelial sodium channel. J Biol Chem (2007) 1.43
Endoplasmic reticulum stress is a determinant of retrovirus-induced spongiform neurodegeneration. J Virol (2003) 1.43
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 1.42
Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation. J Cell Biol (2002) 1.42