Protein aggregation and neurodegenerative disease (Q28273600)

Europe PubMed Central

title

Protein aggregation and neurodegenerative disease (English)

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27 March 2017

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1

Christopher A Ross

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27 March 2017

author name string

Michelle A Poirier

series ordinal

2

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27 March 2017

language of work or name

English

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publication date

July 2004

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27 March 2017

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27 March 2017

volume

10 Suppl

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10

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27 March 2017

page(s)

S10-7

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S10-S17

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27 March 2017

issue

7

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Toxic proteins in neurodegenerative disease

27 March 2017

cites work

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7 January 2021

Huntingtin aggregation and toxicity in Huntington's disease

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7 January 2021

Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders

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7 January 2021

Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration

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7 January 2021

The ubiquitin-proteasome pathway in Parkinson's disease and other neurodegenerative diseases

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7 January 2021

Alzheimer's disease and Parkinson's disease

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7 January 2021

Genetically engineered mouse models of neurodegenerative diseases

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7 January 2021

When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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7 January 2021

Folding proteins in fatal ways

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7 January 2021

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

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7 January 2021

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

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7 January 2021

Neuropathological classification of Huntington's disease

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7 January 2021

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

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7 January 2021

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

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7 January 2021

Clinical and neuropathologic assessment of severity in Huntington's disease

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7 January 2021

Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

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7 January 2021

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

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7 January 2021

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

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7 January 2021

Expansion explosion: new clues to the pathogenesis of repeat expansion neurodegenerative diseases

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7 January 2021

SCA1 molecular genetics: a history of a 13 year collaboration against glutamines

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7 January 2021

Role of histidine interruption in mitigating the pathological effects of long polyglutamine stretches in SCA1: A molecular approach

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7 January 2021

Alzheimer's disease is a synaptic failure

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7 January 2021

The amyloid hypothesis of Alzheimer's disease: progress and problems on the road to therapeutics

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7 January 2021

Direct visualisation of the beta-sheet structure of synthetic Alzheimer's amyloid

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7 January 2021

A portrait of Alzheimer secretases--new features and familiar faces

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7 January 2021

Alzheimer's disease: treatments in discovery and development

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7 January 2021

Tau protein and neurodegeneration

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7 January 2021

Tau gene mutations: dissecting the pathogenesis of FTDP-17.

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7 January 2021

Neuropathology of Parkinson's disease

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7 January 2021

Rare genetic mutations shed light on the pathogenesis of Parkinson disease

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7 January 2021

Hereditary early-onset Parkinson's disease caused by mutations in PINK1

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7 January 2021

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

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7 January 2021

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

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7 January 2021

Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis

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7 January 2021

Shattuck lecture--neurodegenerative diseases and prions

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7 January 2021

Investigating protein conformation-based inheritance and disease in yeast

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7 January 2021

The elongation of yeast prion fibers involves separable steps of association and conversion

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7 January 2021

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

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7 January 2021

Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol

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7 January 2021

X-ray diffraction studies on amyloid filaments

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7 January 2021

From the globular to the fibrous state: protein structure and structural conversion in amyloid formation.

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7 January 2021

Propagating structure of Alzheimer's beta-amyloid(10-35) is parallel beta-sheet with residues in exact register

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7 January 2021

Insights into the amyloid folding problem from solid-state NMR

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7 January 2021

Structural and dynamic features of Alzheimer's Abeta peptide in amyloid fibrils studied by site-directed spin labeling.

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7 January 2021

Structural organization of alpha-synuclein fibrils studied by site-directed spin labeling

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7 January 2021

Amyloid fibril formation by A beta 16-22, a seven-residue fragment of the Alzheimer's beta-amyloid peptide, and structural characterization by solid state NMR

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7 January 2021

Mapping abeta amyloid fibril secondary structure using scanning proline mutagenesis

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7 January 2021

Mutational analysis of the structural organization of polyglutamine aggregates

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7 January 2021

Polyglutamine fibrillogenesis: the pathway unfolds

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7 January 2021

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

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7 January 2021

Conformational Abs recognizing a generic amyloid fibril epitope

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7 January 2021

Protein folding revisited. A polypeptide chain at the folding ? misfolding ? nonfolding cross-roads: which way to go?

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7 January 2021

A one-hit model of cell death in inherited neuronal degenerations

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7 January 2021

Principles of protein folding, misfolding and aggregation

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7 January 2021

Therapeutic strategies for human amyloid diseases

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7 January 2021

Structural neurology: are seeds at the root of neuronal degeneration?

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7 January 2021

Unfolding the role of protein misfolding in neurodegenerative diseases

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7 January 2021

alpha-Synuclein locus triplication causes Parkinson's disease

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7 January 2021

The law of mass action applied to neurodegenerative disease: a hypothesis concerning the etiology and pathogenesis of complex diseases

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7 January 2021

Kinetic stabilization of the alpha-synuclein protofibril by a dopamine-alpha-synuclein adduct

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7 January 2021

Constitutive phosphorylation of the Parkinson's disease associated alpha-synuclein

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7 January 2021

Alpha-synuclein in Lewy bodies

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7 January 2021

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice

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7 January 2021

SUMO modification of Huntingtin and Huntington's disease pathology

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7 January 2021

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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7 January 2021

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

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7 January 2021

Nuclear targeting of mutant Huntingtin increases toxicity

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7 January 2021

Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length.

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7 January 2021

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

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7 January 2021

Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

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7 January 2021

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

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7 January 2021

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

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7 January 2021

Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity

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7 January 2021

Phthalocyanine tetrasulfonates affect the amyloid formation and cytotoxicity of alpha-synuclein

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7 January 2021

Diseases of protein conformation: what do in vitro experiments tell us about in vivo diseases?

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7 January 2021

Ideas of order for amyloid fibril structure

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7 January 2021

Atomic force microscopic imaging of seeded fibril formation and fibril branching by the Alzheimer's disease amyloid-beta protein.

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7 January 2021

Diffusible, nonfibrillar ligands derived from Abeta1-42 are potent central nervous system neurotoxins

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7 January 2021

Assembly of A beta amyloid protofibrils: an in vitro model for a possible early event in Alzheimer's disease.

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7 January 2021

Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins

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7 January 2021

Amyloid beta-protein fibrillogenesis. Detection of a protofibrillar intermediate.

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7 January 2021

Targeting small Abeta oligomers: the solution to an Alzheimer's disease conundrum?

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7 January 2021

Physical basis of cognitive alterations in alzheimer's disease: Synapse loss is the major correlate of cognitive impairment

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7 January 2021

Soluble pool of Abeta amyloid as a determinant of severity of neurodegeneration in Alzheimer's disease

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7 January 2021

Soluble amyloid beta peptide concentration as a predictor of synaptic change in Alzheimer's disease

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7 January 2021

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

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7 January 2021

Vesicle permeabilization by protofibrillar alpha-synuclein: implications for the pathogenesis and treatment of Parkinson's disease.

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7 January 2021

The formation of highly soluble oligomers of alpha-synuclein is regulated by fatty acids and enhanced in Parkinson's disease

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7 January 2021

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

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7 January 2021

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

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7 January 2021

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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7 January 2021

Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein

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7 January 2021

Impairment of the ubiquitin-proteasome system by protein aggregation

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7 January 2021

Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis

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7 January 2021

Molecular chaperones and the art of recognizing a lost cause

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7 January 2021

Protein degradation and protection against misfolded or damaged proteins

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7 January 2021

The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg.

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7 January 2021

Aggresomes, inclusion bodies and protein aggregation

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7 January 2021

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

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7 January 2021

Aggresomes formed by alpha-synuclein and synphilin-1 are cytoprotective

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7 January 2021

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

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7 January 2021

Geldanamycin restores a defective heat shock response in vivo

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7 January 2021

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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7 January 2021

Molecular medicine for the brain: silencing of disease genes with RNA interference

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7 January 2021

Allele-specific silencing of dominant disease genes

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7 January 2021

Immunotherapeutic Approaches to Alzheimer's Disease

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7 January 2021

Good and Bad Amyloid Antibodies

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7 January 2021

Inflammation and therapeutic vaccination in CNS diseases

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7 January 2021

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

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7 January 2021

Self-assembly of beta-amyloid 42 is retarded by small molecular ligands at the stage of structural intermediates

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7 January 2021

Inhibition of beta-amyloid-induced neurotoxicity by imidazopyridoindoles derived from a synthetic combinatorial library

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7 January 2021

Potent inhibition of scrapie prion replication in cultured cells by bis-acridines

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7 January 2021

Modulation of prion protein oligomerization, aggregation, and beta-sheet conversion by 4,4'-dianilino-1,1'-binaphthyl-5,5'-sulfonate (bis-ANS).

1 reference

7 January 2021

Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

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7 January 2021

A rapid cellular FRET assay of polyglutamine aggregation identifies a novel inhibitor.

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7 January 2021

Human β-Secretase (BACE) and BACE Inhibitors

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7 January 2021

A structural model for Alzheimer's beta -amyloid fibrils based on experimental constraints from solid state NMR

1 reference

7 January 2021

Identifiers

DOI

10.1038/NM1066

2 references

27 March 2017

Consolidated OpenCitations Corpus – April 2017

Dimensions Publication ID

1488348

1046979009

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1488348

1 reference

Consolidated OpenCitations Corpus – April 2017

Consolidated OpenCitations Corpus – April 2017

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