Prions and Protein Assemblies that Convey Biological Information in Health and Disease.

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Prions and Protein Assemblies that Convey Biological Information in Health and Disease. is …
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review articleQ7318358
scholarly articleQ13442814

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P356DOI10.1016/J.NEURON.2016.01.026
P932PMC publication ID4748384
P698PubMed publication ID26844828
P5875ResearchGate publication ID292989211

P50authorDavid W SandersQ50857733
Brandon B HolmesQ85308707
P2093author name stringMarc I Diamond
Sarah K Kaufman
P2860cites workThe RIP1/RIP3 necrosome forms a functional amyloid signaling complex required for programmed necrosisQ24293754
MAVS forms functional prion-like aggregates to activate and propagate antiviral innate immune responseQ24316320
Unified polymerization mechanism for the assembly of ASC-dependent inflammasomesQ24339219
Propagation of the yeast prion-like [psi+] determinant is mediated by oligomerization of the SUP35-encoded polypeptide chain release factorQ24562896
Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteinsQ24564014
Altered ribostasis: RNA-protein granules in degenerative disordersQ24604643
Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosisQ24605104
Paired helical filaments from Alzheimer disease brain induce intracellular accumulation of Tau protein in aggresomesQ24608787
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cellsQ24610255
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP functionQ24629019
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALSQ24629495
Heparan sulfate proteoglycans mediate internalization and propagation of specific proteopathic seedsQ24630644
Exome sequencing reveals VCP mutations as a cause of familial ALSQ24631513
PrionsQ24633319
A mesoscale connectome of the mouse brainQ34413358
Heat‐shock protein 70 modulates toxic extracellular α‐synuclein oligomers and rescues trans‐synaptic toxicityQ34421765
Cleavage of tau by asparagine endopeptidase mediates the neurofibrillary pathology in Alzheimer's disease.Q34473854
Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregationQ34488383
Staging of Alzheimer disease-associated neurofibrillary pathology using paraffin sections and immunocytochemistryQ34556945
A novel in vivo model of tau propagation with rapid and progressive neurofibrillary tangle pathology: the pattern of spread is determined by connectivity, not proximityQ34616539
Suicidal [PSI+] is a lethal yeast prionQ34750018
Dopamine promotes formation and secretion of non-fibrillar alpha-synuclein oligomers.Q34901494
Transfer of human α-synuclein from the olfactory bulb to interconnected brain regions in miceQ34909750
Prions hijack tunnelling nanotubes for intercellular spreadQ34938949
In vivo microdialysis reveals age-dependent decrease of brain interstitial fluid tau levels in P301S human tau transgenic miceQ34985153
Differential induction and spread of tau pathology in young PS19 tau transgenic mice following intracerebral injections of pathological tau from Alzheimer's disease or corticobasal degeneration brainsQ35009550
Genomic mosaicism with increased amyloid precursor protein (APP) gene copy number in single neurons from sporadic Alzheimer's disease brains.Q35111229
Ectosomes: a new mechanism for non-exosomal secretion of tau proteinQ35196319
Structural determinants of phenotypic diversity and replication rate of human prionsQ35416860
Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes.Q35539802
Α-synuclein immunotherapy blocks uptake and templated propagation of misfolded α-synuclein and neurodegenerationQ35542169
Molecular and clinical classification of human prion diseaseQ35549016
Templated misfolding of Tau by prion-like seeding along neuronal connections impairs neuronal network function and associated behavioral outcomes in Tau transgenic miceQ35622023
A yeast functional screen predicts new candidate ALS disease genesQ35641365
Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cellsQ35695216
Tau Trimers Are the Minimal Propagation Unit Spontaneously Internalized to Seed Intracellular Aggregation.Q35721664
The disordered P granule protein LAF-1 drives phase separation into droplets with tunable viscosity and dynamicsQ35740324
Human Hsp70 Disaggregase Reverses Parkinson's-Linked α-Synuclein Amyloid FibrilsQ35753373
Exosome-associated tau is secreted in tauopathy models and is selectively phosphorylated in cerebrospinal fluid in early Alzheimer diseaseQ35763138
Highly neurotoxic monomeric α-helical prion proteinQ35779305
Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in miceQ35946149
Tau pathology spread in PS19 tau transgenic mice following locus coeruleus (LC) injections of synthetic tau fibrils is determined by the LC's afferent and efferent connectionsQ35985428
Predicting regional neurodegeneration from the healthy brain functional connectomeQ35991719
Transgene expression in the Nop-tTA driver line is not inherently restricted to the entorhinal cortexQ36161795
Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding ProteinsQ36172705
Modifiers of solid RNP granules control normal RNP dynamics and mRNA activity in early developmentQ36266144
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule FunctionQ36320469
Cells release prions in association with exosomesQ36448448
Biochemical classification of tauopathies by immunoblot, protein sequence and mass spectrometric analyses of sarkosyl-insoluble and trypsin-resistant tauQ36465091
Extracellular Tau levels are influenced by variability in Tau that is associated with tauopathiesQ36466732
Structural variants of yeast prions show conformer-specific requirements for chaperone activity.Q36498591
Small misfolded Tau species are internalized via bulk endocytosis and anterogradely and retrogradely transported in neuronsQ36543778
Synthetic tau fibrils mediate transmission of neurofibrillary tangles in a transgenic mouse model of Alzheimer's-like tauopathyQ36617500
Prion-like spreading of pathological α-synuclein in brainQ36732844
Stress granules as crucibles of ALS pathogenesis.Q36804082
Brain homogenates from human tauopathies induce tau inclusions in mouse brainQ36915421
Activation of asparaginyl endopeptidase leads to Tau hyperphosphorylation in Alzheimer diseaseQ36929023
Caudo-rostral brain spreading of α-synuclein through vagal connectionsQ37040842
Reversal of neurofibrillary tangles and tau-associated phenotype in the rTgTauEC model of early Alzheimer's diseaseQ37093852
Identification of an intracellular site of prion conversionQ37170450
Stages of pTDP-43 pathology in amyotrophic lateral sclerosisQ37202139
Mechanism of tau-induced neurodegeneration in Alzheimer disease and related tauopathiesQ37239077
Structural and functional characterization of two alpha-synuclein strainsQ37305236
Cell-produced alpha-synuclein is secreted in a calcium-dependent manner by exosomes and impacts neuronal survivalQ37347595
Transmission of multiple system atrophy prions to transgenic miceQ37353012
Natural and synthetic prion structure from X-ray fiber diffractionQ37386047
Translation repressors, an RNA helicase, and developmental cues control RNP phase transitions during early development.Q37407831
Comparative survey of the topographical distribution of signature molecular lesions in major neurodegenerative diseasesQ37412619
Exogenous alpha-synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cellsQ37446544
Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.Q37451385
Virus entry by macropinocytosisQ37464753
RNA Controls PolyQ Protein Phase Transitions.Q37573060
Neuronal activity regulates extracellular tau in vivoQ37630201
ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arborQ37650180
The transcellular spread of cytosolic amyloids, prions, and prionoidsQ37671889
Neuropathology underlying clinical variability in patients with synucleinopathiesQ37896515
α-Synuclein posttranslational modification and alternative splicing as a trigger for neurodegenerationQ38038123
Cellular mechanisms of protein aggregate propagationQ38056356
Stress granules and cell signaling: more than just a passing phase?Q38136844
Demystifying heparan sulfate-protein interactions.Q38194193
Nuclear bodies: new insights into assembly/dynamics and disease relevance.Q38202450
Liquid-liquid phase separation in biologyQ38257388
Extracellular monomeric tau protein is sufficient to initiate the spread of tau protein pathologyQ39062953
The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analogQ24643343
Transmission and spreading of tauopathy in transgenic mouse brainQ24651334
Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synucleinQ24655848
Tau fragmentation, aggregation and clearance: the dual role of lysosomal processingQ24656204
Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregatesQ24657543
A systematic survey identifies prions and illuminates sequence features of prionogenic proteinsQ24658451
Formation of native prions from minimal components in vitroQ24676353
Mechanism of prion propagation: amyloid growth occurs by monomer additionQ24793082
Propagation of prions causing synucleinopathies in cultured cellsQ26269822
Phase transitions and size scaling of membrane-less organellesQ26852037
Prion-like polymerization as a signaling mechanismQ26995776
Neurodegenerative diseases: expanding the prion conceptQ27011744
The structure of ClpB: a molecular chaperone that rescues proteins from an aggregated stateQ27642377
Molecular Structure of β-Amyloid Fibrils in Alzheimer’s Disease Brain TissueQ27679935
Structural basis for the prion-like MAVS filaments in antiviral innate immunityQ27681906
Neuropathological stageing of Alzheimer-related changesQ27860862
Identification and characterization of MAVS, a mitochondrial antiviral signaling protein that activates NF-kappaB and IRF 3Q27861127
Hsp104, Hsp70, and Hsp40: a novel chaperone system that rescues previously aggregated proteinsQ27931364
Spreading of pathology in neurodegenerative diseases: a focus on human studiesQ28082279
Staging of brain pathology related to sporadic Parkinson's diseaseQ28131702
A yeast prion provides a mechanism for genetic variation and phenotypic diversityQ28140932
A neuronal isoform of CPEB regulates local protein synthesis and stabilizes synapse-specific long-term facilitation in aplysiaQ28236378
[URE3] as an altered URE2 protein: evidence for a prion analog in Saccharomyces cerevisiaeQ28237493
The NLRP3 inflammasome is released as a particulate danger signal that amplifies the inflammatory responseQ28242527
The adaptor ASC has extracellular and 'prionoid' activities that propagate inflammationQ28242537
A general model of prion strains and their pathogenicityQ28256770
Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and hostQ28264821
Protein aggregation and neurodegenerative diseaseQ28273600
Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson's diseaseQ28275257
Dissociable intrinsic connectivity networks for salience processing and executive controlQ28290658
Distinct α-synuclein strains differentially promote tau inclusions in neuronsQ28293967
The neuropathology of sportQ28383563
Conformational variations in an infectious protein determine prion strain differencesQ44187524
Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brainQ44568749
Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformersQ44904974
Focality of upper and lower motor neuron degeneration at the clinical onset of ALS.Q44929155
Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortemQ44929159
Transfer of polyglutamine aggregates in neuronal cells occurs in tunneling nanotubesQ45080144
Scrapie in the central nervous system: neuroanatomical spread of infection and Sinc control of pathogenesisQ45226126
The physical basis of how prion conformations determine strain phenotypesQ46092862
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversityQ46433690
Lewy body extracts from Parkinson disease brains trigger α-synuclein pathology and neurodegeneration in mice and monkeys.Q46970748
Germline P granules are liquid droplets that localize by controlled dissolution/condensationQ47748097
Cerebrospinal fluid tau as a marker of neuronal damage after epileptic seizureQ47844905
On the identification of low allele frequency mosaic mutations in the brains of Alzheimer's disease patientsQ48195772
Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected miceQ48289964
Targeting of scrapie lesions and spread of agent via the retino-tectal projectionQ48444310
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob diseaseQ48691137
Scrapie in mice. Agent-strain differences in the distribution and intensity of grey matter vacuolationQ48707789
Identification of a protein that purifies with the scrapie prionQ48858653
Transient increase in total tau but not phospho-tau in human cerebrospinal fluid after acute strokeQ49117210
A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation.Q53368534
Constitutive secretion of tau protein by an unconventional mechanism.Q54306723
Epigenetic regulation of translation reveals hidden genetic variation to produce complex traitsQ59049636
The microtubule-associated protein tau forms a triple-stranded left-hand helical polymerQ68041188
Lysosomal enzyme cathepsin B enhances the aggregate forming activity of exogenous α-synuclein fibrilsQ86168750
A comparison of some biological characteristics of the mouse-passaged scrapie agents, 22A and ME7Q93789088
The Persistence of Hippocampal-Based Memory Requires Protein Synthesis Mediated by the Prion-like Protein CPEB3Q28586955
Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillizationQ28588090
Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's diseaseQ28588314
Trans-synaptic spread of tau pathology in vivoQ28732288
A Network Diffusion Model of Disease Progression in DementiaQ29543923
Parkinson's disease: mechanisms and modelsQ29547424
Novel proteinaceous infectious particles cause scrapieQ29547678
Neurodegenerative diseases target large-scale human brain networksQ29614723
Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogelsQ29614781
Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagationQ29615640
Resting-state functional connectivity reflects structural connectivity in the default mode networkQ29616527
Eight prion strains have PrP(Sc) molecules with different conformationsQ29617277
Self-propagating, molecular-level polymorphism in Alzheimer's beta-amyloid fibrilsQ29617476
Intrinsic functional architecture in the anaesthetized monkey brainQ29619207
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing proteinQ29619232
Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [psi+]Q29619693
Neurodegenerative tauopathiesQ29619895
Propagation of tau pathology in a model of early Alzheimer's diseaseQ29620203
Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic miceQ29620597
Heparan sulphate proteoglycan and the low-density lipoprotein receptor-related protein 1 constitute major pathways for neuronal amyloid-beta uptakeQ29976923
Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy.Q30420123
Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutationsQ30573122
Phosphorylation-regulated binding of RNA polymerase II to fibrous polymers of low-complexity domainsQ30577537
The multivesicular body is the major internal site of prion conversionQ30633753
Pathologic prion protein infects cells by lipid-raft dependent macropinocytosisQ33434501
Isolation of a fragment of tau derived from the core of the paired helical filament of Alzheimer diseaseQ33582860
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP diseaseQ33589965
Specific calpain inhibition by calpastatin prevents tauopathy and neurodegeneration and restores normal lifespan in tau P301L miceQ33865294
Neuron-released oligomeric α-synuclein is an endogenous agonist of TLR2 for paracrine activation of microgliaQ33874305
Search for a prion-specific nucleic acidQ33911902
A neuronal isoform of the aplysia CPEB has prion-like propertiesQ33974852
The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free systemQ34056277
Sensitive detection of pathological prion protein by cyclic amplification of protein misfoldingQ34084209
Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation.Q34097850
Pathological alpha-synuclein propagates through neural networksQ34100338
Amyloid structure: conformational diversity and consequencesQ34175001
The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Q34178673
Experimental transmission of a Kuru-like syndrome to chimpanzeesQ34241831
Prions are a common mechanism for phenotypic inheritance in wild yeasts.Q34254667
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.Q34263380
Hyperphosphorylation and cleavage at D421 enhance tau secretionQ34277477
Metazoan Hsp70 machines use Hsp110 to power protein disaggregationQ34300523
Higher-order assemblies in a new paradigm of signal transductionQ34338941
Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C).Q34372311
Evidence for assembly of prions with left-handed beta-helices into trimersQ34375245
Proteopathic tau seeding predicts tauopathy in vivo.Q34383788
Support for the prion hypothesis for inheritance of a phenotypic trait in yeastQ34384236
Chaperone-mediated autophagy: roles in disease and agingQ34388237
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD.Q34403513
Prion-like polymerization underlies signal transduction in antiviral immune defense and inflammasome activationQ34410101
The seeds of neurodegeneration: prion-like spreading in ALSQ39247056
Cell-free formation of RNA granules: bound RNAs identify features and components of cellular assemblies.Q39349822
Extensive diversity of prion strains is defined by differential chaperone interactions and distinct amyloidogenic regions.Q39385432
Proteostasis of tau. Tau overexpression results in its secretion via membrane vesicles.Q39433741
Physiological release of endogenous tau is stimulated by neuronal activity.Q39560268
Interneuronal transfer of human tau between Lamprey central neurons in situQ39746642
Propagation of tau misfolding from the outside to the inside of a cell.Q39873721
Intravesicular localization and exocytosis of alpha-synuclein and its aggregates.Q40405368
Staging of Alzheimer's disease-related neurofibrillary changesQ40416705
Heparan sulfate is a cellular receptor for purified infectious prions.Q40466338
Scrapie strain variation and mutationQ40729976
Accumulation of vesicle-associated human tau in distal dendrites drives degeneration and tau secretion in an in situ cellular tauopathy modelQ40796484
Internalization of transthyretin. Evidence of a novel yet unidentified receptor-associated protein (RAP)-sensitive receptorQ40816732
The CPEB3 Protein Is a Functional Prion that Interacts with the Actin Cytoskeleton.Q40836097
Phase transition of a disordered nuage protein generates environmentally responsive membraneless organellesQ41277254
Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da.Q41388682
α-Synuclein strains cause distinct synucleinopathies after local and systemic administrationQ41681113
Evolutionary link between metazoan RHIM motif and prion-forming domain of fungal heterokaryon incompatibility factor HET-s/HET-sQ41698579
Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathiesQ41760966
The structural basis of yeast prion strain variantsQ41792123
Genesis and variability of [PSI] prion factors in Saccharomyces cerevisiae.Q41815189
Distinct tau prion strains propagate in cells and mice and define different tauopathiesQ41904960
Trimeric tau is toxic to human neuronal cells at low nanomolar concentrationsQ42042649
Conformational diversity of wild-type Tau fibrils specified by templated conformation changeQ42114209
Determinants of amyloid fibril degradation by the PDZ protease HTRA1.Q42152333
Heritable remodeling of yeast multicellularity by an environmentally responsive prionQ42263660
Starvation and inhibition of lysosomal function increased tau secretion by primary cortical neuronsQ42738229
Seeded strain-like transmission of β-amyloid morphotypes in APP transgenic miceQ42921084
Conformation determines the seeding potencies of native and recombinant Tau aggregatesQ43106197
Intracerebral injection of preformed synthetic tau fibrils initiates widespread tauopathy and neuronal loss in the brains of tau transgenic miceQ43116153
Critical role of amyloid-like oligomers of Drosophila Orb2 in the persistence of memoryQ43529889
P433issue3
P407language of work or nameEnglishQ1860
P921main subjectprion protein familyQ24724413
P304page(s)433-448
P577publication date2016-02-01
P1433published inNeuronQ3338676
P1476titlePrions and Protein Assemblies that Convey Biological Information in Health and Disease
P478volume89