Protein aggregation diseases: pathogenicity and therapeutic perspectives (Q34101110)

31 July 2017

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title

Protein aggregation diseases: pathogenicity and therapeutic perspectives (English)

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31 July 2017

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Adriano Aguzzi

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Tracy O'Connor

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31 July 2017

publication date

1 March 2010

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Nature Reviews Drug Discovery

31 July 2017

published in

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31 July 2017

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31 July 2017

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31 July 2017

page(s)

237-248

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Atomic structures of amyloid cross-beta spines reveal varied steric zippers

31 July 2017

cites work

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Ueber eine im Gehirn und Rückenmark des Menschen aufgefundene Substanz mit der chemischen Reaction der Cellulose

31 March 2020

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Aggregation propensity of the human proteome

31 March 2020

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Islet amyloid: a critical entity in the pathogenesis of type 2 diabetes

31 March 2020

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Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis

31 March 2020

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Aspects on human amyloid forms and their fibril polypeptides

31 March 2020

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The fold of alpha-synuclein fibrils

31 March 2020

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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

31 March 2020

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Amyloid beta-protein fibrillogenesis. Structure and biological activity of protofibrillar intermediates

31 March 2020

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Soluble protein oligomers as emerging toxins in Alzheimer's and other amyloid diseases.

31 March 2020

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Intraneuronal beta-amyloid aggregates, neurodegeneration, and neuron loss in transgenic mice with five familial Alzheimer's disease mutations: potential factors in amyloid plaque formation.

31 March 2020

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Massive CA1/2 neuronal loss with intraneuronal and N-terminal truncated Abeta42 accumulation in a novel Alzheimer transgenic model

31 March 2020

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Amyloid plaque core protein in Alzheimer disease and Down syndrome

31 March 2020

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Twenty years of the Alzheimer's disease amyloid hypothesis: a genetic perspective

31 March 2020

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Therapeutic strategies for Alzheimer's disease

31 March 2020

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Hope in Alzheimer's fight emerges from unexpected places

31 March 2020

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Games played by rogue proteins in prion disorders and Alzheimer's disease

31 March 2020

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Functional gamma-secretase inhibitors reduce beta-amyloid peptide levels in brain

31 March 2020

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Dynamics of {beta}-amyloid reductions in brain, cerebrospinal fluid, and plasma of {beta}-amyloid precursor protein transgenic mice treated with a {gamma}-secretase inhibitor

31 March 2020

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Quantitative measurement of changes in amyloid-beta(40) in the rat brain and cerebrospinal fluid following treatment with the gamma-secretase inhibitor LY-411575 [N2-[(2S)-2-(3,5-difluorophenyl)-2-hydroxyethanoyl]-N1-[(7S)-5-methyl-6-oxo-6,7-dihydro

31 March 2020

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Effects of a gamma-secretase inhibitor in a randomized study of patients with Alzheimer disease

31 March 2020

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Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein

31 March 2020

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A presenilin-1-dependent gamma-secretase-like protease mediates release of Notch intracellular domain

31 March 2020

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Presenilin 1 is required for Notch1 and DII1 expression in the paraxial mesoderm

31 March 2020

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Skeletal and CNS defects in Presenilin-1-deficient mice

31 March 2020

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Chronic treatment with the gamma-secretase inhibitor LY-411,575 inhibits beta-amyloid peptide production and alters lymphopoiesis and intestinal cell differentiation

31 March 2020

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Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease

31 March 2020

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Candidate gene for the chromosome 1 familial Alzheimer's disease locus

31 March 2020

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Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene

31 March 2020

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Increased amyloid-beta42(43) in brains of mice expressing mutant presenilin 1

31 March 2020

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The E280A presenilin 1 Alzheimer mutation produces increased A beta 42 deposition and severe cerebellar pathology

31 March 2020

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Mutant presenilins of Alzheimer's disease increase production of 42-residue amyloid beta-protein in both transfected cells and transgenic mice

31 March 2020

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A subset of NSAIDs lower amyloidogenic Abeta42 independently of cyclooxygenase activity

31 March 2020

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gamma-Secretase substrate selectivity can be modulated directly via interaction with a nucleotide-binding site

31 March 2020

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1-(3',4'-Dichloro-2-fluoro[1,1'-biphenyl]-4-yl)-cyclopropanecarboxylic acid (CHF5074), a novel gamma-secretase modulator, reduces brain beta-amyloid pathology in a transgenic mouse model of Alzheimer's disease without causing peripheral toxicity.

31 March 2020

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Chronic administration of R-flurbiprofen attenuates learning impairments in transgenic amyloid precursor protein mice

31 March 2020

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Safety, tolerability, pharmacokinetics, and Abeta levels after short-term administration of R-flurbiprofen in healthy elderly individuals

31 March 2020

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Efficacy and safety of tarenflurbil in mild to moderate Alzheimer's disease: a randomised phase II trial

31 March 2020

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The orphan G protein-coupled receptor 3 modulates amyloid-beta peptide generation in neurons

31 March 2020

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gamma-Secretase heterogeneity in the Aph1 subunit: relevance for Alzheimer's disease

31 March 2020

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Beta-secretase cleavage of Alzheimer's amyloid precursor protein by the transmembrane aspartic protease BACE

31 March 2020

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Identification of a Novel Aspartic Protease (Asp 2) as β-Secretase

31 March 2020

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Human aspartic protease memapsin 2 cleaves the beta-secretase site of beta-amyloid precursor protein

31 March 2020

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Purification and cloning of amyloid precursor protein beta-secretase from human brain

31 March 2020

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Membrane-anchored aspartyl protease with Alzheimer's disease beta-secretase activity

31 March 2020

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Mice deficient in BACE1, the Alzheimer's beta-secretase, have normal phenotype and abolished beta-amyloid generation

31 March 2020

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BACE1 deficiency rescues memory deficits and cholinergic dysfunction in a mouse model of Alzheimer's disease

31 March 2020

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BACE1 gene deletion prevents neuron loss and memory deficits in 5XFAD APP/PS1 transgenic mice

31 March 2020

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BACE1, a major determinant of selective vulnerability of the brain to amyloid-beta amyloidogenesis, is essential for cognitive, emotional, and synaptic functions.

31 March 2020

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Partial reduction of BACE1 has dramatic effects on Alzheimer plaque and synaptic pathology in APP Transgenic Mice

31 March 2020

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Memapsin 2 (beta-secretase) inhibitors: drug development

31 March 2020

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Bace1 modulates myelination in the central and peripheral nervous system.

31 March 2020

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BACE1 regulates voltage-gated sodium channels and neuronal activity

31 March 2020

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In vivo cleavage of alpha2,6-sialyltransferase by Alzheimer beta-secretase

31 March 2020

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Regulated intramembrane proteolysis of the interleukin-1 receptor II by alpha-, beta-, and gamma-secretase

31 March 2020

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Cleavage of amyloid-beta precursor protein and amyloid-beta precursor-like protein by BACE 1

31 March 2020

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The cell adhesion protein P-selectin glycoprotein ligand-1 is a substrate for the aspartyl protease BACE1.

31 March 2020

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BACE (beta-secretase) modulates the processing of APLP2 in vivo

31 March 2020

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The low density lipoprotein receptor-related protein (LRP) is a novel beta-secretase (BACE1) substrate.

31 March 2020

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beta-site amyloid precursor protein cleaving enzyme 1 increases amyloid deposition in brain parenchyma but reduces cerebrovascular amyloid angiopathy in aging BACE x APP[V717I] double-transgenic mice

31 March 2020

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beta Subunits of voltage-gated sodium channels are novel substrates of beta-site amyloid precursor protein-cleaving enzyme (BACE1) and gamma-secretase

31 March 2020

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An update on treatment and prevention strategies for Alzheimer's disease

31 March 2020

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Phosphorylation of the translation initiation factor eIF2alpha increases BACE1 levels and promotes amyloidogenesis

31 March 2020

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Transcriptional regulation of beta-secretase by p25/cdk5 leads to enhanced amyloidogenic processing

31 March 2020

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Loss of microRNA cluster miR-29a/b-1 in sporadic Alzheimer's disease correlates with increased BACE1/beta-secretase expression

31 March 2020

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The expression of microRNA miR-107 decreases early in Alzheimer's disease and may accelerate disease progression through regulation of beta-site amyloid precursor protein-cleaving enzyme 1.

31 March 2020

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Expression of a noncoding RNA is elevated in Alzheimer's disease and drives rapid feed-forward regulation of beta-secretase

31 March 2020

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Depletion of GGA3 stabilizes BACE and enhances beta-secretase activity

31 March 2020

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Reticulon family members modulate BACE1 activity and amyloid-beta peptide generation

31 March 2020

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Efficient inhibition of the Alzheimer's disease beta-secretase by membrane targeting

31 March 2020

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Immunization with amyloid-beta attenuates Alzheimer-disease-like pathology in the PDAPP mouse

31 March 2020

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A beta peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer's disease

31 March 2020

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A beta peptide vaccination prevents memory loss in an animal model of Alzheimer's disease

31 March 2020

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Immunization with a nontoxic/nonfibrillar amyloid-beta homologous peptide reduces Alzheimer's disease-associated pathology in transgenic mice

31 March 2020

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Peripherally administered antibodies against amyloid beta-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer disease

31 March 2020

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Peripheral anti-A beta antibody alters CNS and plasma A beta clearance and decreases brain A beta burden in a mouse model of Alzheimer's disease

31 March 2020

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Brain to plasma amyloid-beta efflux: a measure of brain amyloid burden in a mouse model of Alzheimer's disease

31 March 2020

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Immunization reverses memory deficits without reducing brain Abeta burden in Alzheimer's disease model

31 March 2020

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Dosing in phase II trial of Alzheimer's vaccine suspended

31 March 2020

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Clinical effects of Abeta immunization (AN1792) in patients with AD in an interrupted trial.

31 March 2020

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Intravenous immunoglobulins containing antibodies against beta-amyloid for the treatment of Alzheimer's disease

31 March 2020

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18-Month study of intravenous immunoglobulin for treatment of mild Alzheimer disease.

31 March 2020

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Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor

31 March 2020

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Amyloid-beta immunotherapy for Alzheimer's disease: the end of the beginning

31 March 2020

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Persistent amyloidosis following suppression of Abeta production in a transgenic model of Alzheimer disease

31 March 2020

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Immunoaffinity purification and neutralization of scrapie prion infectivity

31 March 2020

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Plaques, tangles and dementia. A quantitative study

31 March 2020

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Aggregation of secreted amyloid beta-protein into sodium dodecyl sulfate-stable oligomers in cell culture

31 March 2020

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The oligomerization of amyloid beta-protein begins intracellularly in cells derived from human brain

31 March 2020

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Alzheimer's disease-affected brain: presence of oligomeric A beta ligands (ADDLs) suggests a molecular basis for reversible memory loss

31 March 2020

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A specific amyloid-beta protein assembly in the brain impairs memory

31 March 2020

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Cyclohexanehexol inhibitors of Abeta aggregation prevent and reverse Alzheimer phenotype in a mouse model.

31 March 2020

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Amyloid-beta protein dimers isolated directly from Alzheimer's brains impair synaptic plasticity and memory

31 March 2020

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Beta-amyloid oligomers induce phosphorylation of tau and inactivation of insulin receptor substrate via c-Jun N-terminal kinase signaling: suppression by omega-3 fatty acids and curcumin.

31 March 2020

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Soluble oligomers of amyloid Beta protein facilitate hippocampal long-term depression by disrupting neuronal glutamate uptake

31 March 2020

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β-Amyloid impairs axonal BDNF retrograde trafficking

31 March 2020

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Insulin receptor dysfunction impairs cellular clearance of neurotoxic oligomeric a{beta}

31 March 2020

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Protection of synapses against Alzheimer's-linked toxins: insulin signaling prevents the pathogenic binding of Abeta oligomers

31 March 2020

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Conformation-dependent anti-amyloid oligomer antibodies

31 March 2020

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Intraneuronal Abeta42 accumulation in human brain.

31 March 2020

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Apolipoprotein E and low density lipoprotein receptor-related protein facilitate intraneuronal Abeta42 accumulation in amyloid model mice

31 March 2020

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Synaptic activity reduces intraneuronal Abeta, promotes APP transport to synapses, and protects against Abeta-related synaptic alterations

31 March 2020

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Long-term neprilysin gene transfer is associated with reduced levels of intracellular Abeta and behavioral improvement in APP transgenic mice

31 March 2020

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Immunotherapy for Alzheimer's disease: attacking amyloid-beta from the inside

31 March 2020

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Immunization with amyloid-beta attenuates inclusion body myositis-like myopathology and motor impairment in a transgenic mouse model

31 March 2020

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Independent effects of intra- and extracellular Abeta on learning-related gene expression

31 March 2020

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Molecular mechanisms of prion pathogenesis

31 March 2020

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Beyond the prion principle

31 March 2020

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A crucial role for B cells in neuroinvasive scrapie.

31 March 2020

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Complement facilitates early prion pathogenesis

31 March 2020

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Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion

31 March 2020

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Lymph nodal prion replication and neuroinvasion in mice devoid of follicular dendritic cells

31 March 2020

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Repetitive immunization enhances the susceptibility of mice to peripherally administered prions

31 March 2020

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Bacterial colitis increases susceptibility to oral prion disease

31 March 2020

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PrPSc in mammary glands of sheep affected by scrapie and mastitis

31 March 2020

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Chronic Lymphocytic Inflammation Specifies the Organ Tropism of Prions

31 March 2020

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Prion pathogenesis in the absence of Toll-like receptor signalling

31 March 2020

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Experimental autoimmune encephalomyelitis repressed by microglial paralysis.

31 March 2020

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Antiprion immunotherapy: to suppress or to stimulate?

31 March 2020

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Impaired prion replication in spleens of mice lacking functional follicular dendritic cells

31 March 2020

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Lymphotoxin-Dependent Prion Replication in Inflammatory Stromal Cells of Granulomas

31 March 2020

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Cells and prions: a license to replicate

31 March 2020

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Immunization delays the onset of prion disease in mice.

31 March 2020

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Decrease in pathology and progression of scrapie after immunisation with synthetic prion protein peptides in hamsters.

31 March 2020

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Humoral immune response to native eukaryotic prion protein correlates with anti-prion protection.

31 March 2020

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Anti-prion antibodies for prophylaxis following prion exposure in mice

31 March 2020

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Modulation of proteinase-K resistant prion protein by prion peptide immunization.

31 March 2020

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Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies

31 March 2020

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Monoclonal antibodies inhibit prion replication and delay the development of prion disease

31 March 2020

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Cross-Linking Cellular Prion Protein Triggers Neuronal Apoptosis in Vivo

31 March 2020

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A systematic review of prion therapeutics in experimental models

31 March 2020

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Molecular dissection of Alzheimer's disease neuropathology by depletion of serum amyloid P component

31 March 2020

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Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation

31 March 2020

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Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease

31 March 2020

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Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models

31 March 2020

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Evaluation of quinacrine treatment for prion diseases

31 March 2020

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Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial

31 March 2020

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Mimicking dominant negative inhibition of prion replication through structure-based drug design

31 March 2020

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Library design, synthesis, and screening: pyridine dicarbonitriles as potential prion disease therapeutics

31 March 2020

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Reversion of prion protein conformational changes by synthetic b-sheet breaker peptides

31 March 2020

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Beta-sheet breaker peptides inhibit fibrillogenesis in a rat brain model of amyloidosis: implications for Alzheimer's therapy

31 March 2020

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Alzheimer A beta neurotoxicity: promotion by antichymotrypsin, ApoE4; inhibition by A beta-related peptides

31 March 2020

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Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosis

31 March 2020

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Prion strain discrimination using luminescent conjugated polymers

31 March 2020

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Pathogenesis of and therapeutic strategies to ameliorate the transthyretin amyloidoses

31 March 2020

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Normal host prion protein necessary for scrapie-induced neurotoxicity

31 March 2020

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Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis.

31 March 2020

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Anchorless prion protein results in infectious amyloid disease without clinical scrapie

31 March 2020

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Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers

31 March 2020

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Signals determining protein tyrosine kinase and glycosyl-phosphatidylinositol-anchored protein targeting to a glycolipid-enriched membrane fraction

31 March 2020

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Sorting and intracellular trafficking of a glycosylphosphatidylinositol-anchored protein and two hybrid transmembrane proteins with the same ectodomain in Madin-Darby canine kidney epithelial cells

31 March 2020

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Lipid domain structure of the plasma membrane revealed by patching of membrane components

31 March 2020

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Differential sorting and fate of endocytosed GPI-anchored proteins

31 March 2020

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Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions

31 March 2020

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Lethal recessive myelin toxicity of prion protein lacking its central domain

31 March 2020

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Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125.

31 March 2020

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3D structure of Alzheimer's amyloid-beta(1-42) fibrils

31 March 2020

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Germinal center B cells are dispensable in prion transport and neuroinvasion

31 March 2020

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AA-amyloidosis can be transferred by peripheral blood monocytes

31 March 2020

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Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility

31 March 2020

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Structural typing of systemic amyloidoses by luminescent-conjugated polymer spectroscopy

31 March 2020

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Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.

31 March 2020

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Differential inhibition of prion propagation by enantiomers of quinacrine

31 March 2020

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12 December 2020

The role of cell-derived oligomers of Abeta in Alzheimer's disease and avenues for therapeutic intervention.

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12 December 2020

Congo red and thioflavin-T analogs detect Abeta oligomers

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12 December 2020

Semagacestat, a gamma-secretase inhibitor for the potential treatment of Alzheimer's disease.

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12 December 2020

Identifiers

DOI

10.1038/NRD3050

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31 July 2017

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