Walking the tightrope: proteostasis and neurodegenerative disease (Q38731221)

5 September 2017

title

Walking the tightrope: proteostasis and neurodegenerative disease (English)

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5 September 2017

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Darren N Saunders

4

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Lezanne Ooi

Lezanne Ooi

2

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Heath Ecroyd

Heath Ecroyd

9

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Mark R. Wilson

Mark R Wilson

8

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Danny M. Hatters

Danny M Hatters

5

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Neil Cashman

7

Neil R Cashman

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5 September 2017

1

Justin J Yerbury

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5 September 2017

Andrew Dillin

3

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5 September 2017

Philip M Beart

6

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5 September 2017

language of work or name

English

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publication date

12 February 2016

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Journal of Neurochemistry

5 September 2017

published in

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5 September 2017

volume

137

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5 September 2017

issue

4

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5 September 2017

page(s)

489-505

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Heat shock factors: integrators of cell stress, development and lifespan.

5 September 2017

cites work

1 reference

Regulation of HSF1 function in the heat stress response: implications in aging and disease

21 January 2018

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Loss of Usp14 results in reduced levels of ubiquitin in ataxia mice

21 January 2018

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Conditioning protects C. elegans from lethal effects of enteropathogenic E. coli by activating genes that regulate lifespan and innate immunity

21 January 2018

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The pathways of mitophagy for quality control and clearance of mitochondria

21 January 2018

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Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.

21 January 2018

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Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease

21 January 2018

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HSF-1-mediated cytoskeletal integrity determines thermotolerance and life span

21 January 2018

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Mitochondrial protein quality control in health and disease

21 January 2018

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Adapting proteostasis for disease intervention

21 January 2018

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Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.

21 January 2018

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Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis.

21 January 2018

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Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging

21 January 2018

1 reference

Ubiquitin editing enzyme UCH L1 and microtubule dynamics: implication in mitosis

21 January 2018

1 reference

Neuronal macroautophagy: from development to degeneration

21 January 2018

1 reference

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function

21 January 2018

1 reference

The Chemical Biology of Molecular Chaperones--Implications for Modulation of Proteostasis

21 January 2018

1 reference

A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease

21 January 2018

1 reference

A systems biology investigation of neurodegenerative dementia reveals a pivotal role of autophagy

21 January 2018

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Protein misfolding, functional amyloid, and human disease

21 January 2018

1 reference

A three-dimensional human neural cell culture model of Alzheimer's disease

21 January 2018

1 reference

Widespread aggregation and neurodegenerative diseases are associated with supersaturated proteins

21 January 2018

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Autophagic cell death exists

21 January 2018

1 reference

Preventing α-synuclein aggregation: the role of the small heat-shock molecular chaperone proteins

21 January 2018

1 reference

A dynamic ubiquitin equilibrium couples proteasomal activity to chromatin remodeling

21 January 2018

1 reference

Heat‐shock protein 70 modulates toxic extracellular α‐synuclein oligomers and rescues trans‐synaptic toxicity

21 January 2018

1 reference

Widespread protein aggregation as an inherent part of aging in C. elegans

21 January 2018

1 reference

Heat shock protein 70 inhibits alpha-synuclein fibril formation via preferential binding to prefibrillar species

21 January 2018

1 reference

The deubiquitinase USP28 controls intestinal homeostasis and promotes colorectal cancer

21 January 2018

1 reference

The role of HSP70 and its co-chaperones in protein misfolding, aggregation and disease.

21 January 2018

1 reference

The molecular chaperone Hsp90 modulates intermediate steps of amyloid assembly of the Parkinson-related protein alpha-synuclein.

21 January 2018

1 reference

Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

21 January 2018

1 reference

Frontotemporal dementia and its subtypes: a genome-wide association study

21 January 2018

1 reference

Multi-layered molecular mechanisms of polypeptide holding, unfolding and disaggregation by HSP70/HSP110 chaperones

21 January 2018

1 reference

Chemical modulators of autophagy as biological probes and potential therapeutics

21 January 2018

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Promoting autophagic clearance: viable therapeutic targets in Alzheimer's disease

21 January 2018

1 reference

Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models

21 January 2018

1 reference

Cmr1/WDR76 defines a nuclear genotoxic stress body linking genome integrity and protein quality control

21 January 2018

1 reference

Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells

21 January 2018

1 reference

Cause and consequence: mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases.

21 January 2018

1 reference

Progressive disruption of cellular protein folding in models of polyglutamine diseases.

21 January 2018

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TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.

21 January 2018

1 reference

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

21 January 2018

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Ubiquitin crosstalk connecting cellular processes

21 January 2018

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Generalization of the prion hypothesis to other neurodegenerative diseases: an imperfect fit.

21 January 2018

1 reference

Caveolin-1-dependent apoptosis induced by fibrin degradation products

21 January 2018

1 reference

Neuronal ubiquitin homeostasis

21 January 2018

1 reference

Cell-free formation of RNA granules: bound RNAs identify features and components of cellular assemblies.

21 January 2018

1 reference

Ubiquitin depletion as a key mediator of toxicity by translational inhibitors

21 January 2018

1 reference

Molecular chaperones in protein folding and proteostasis

21 January 2018

1 reference

Ubiquitin-dependent mitochondrial protein degradation

21 January 2018

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Proposed role of ATP in protein breakdown: conjugation of protein with multiple chains of the polypeptide of ATP-dependent proteolysis

21 January 2018

1 reference

Oxidative stress: emerging mitochondrial and cellular themes and variations in neuronal injury.

21 January 2018

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Autophagic activity in cortical neurons under acute oxidative stress directly contributes to cell death.

21 January 2018

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Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease

21 January 2018

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Heat shock protein-inducing compounds as therapeutics to restore proteostasis in atrial fibrillation

21 January 2018

1 reference

Suppression of Alzheimer's disease-related phenotypes by expression of heat shock protein 70 in mice.

21 January 2018

1 reference

Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin.

21 January 2018

1 reference

Negative regulation of 26S proteasome stability via calpain-mediated cleavage of Rpn10 subunit upon mitochondrial dysfunction in neurons

21 January 2018

1 reference

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1α.

21 January 2018

1 reference

GRP78 counteracts cell death and protein aggregation caused by mutant huntingtin proteins

21 January 2018

1 reference

Aggresomes: a cellular response to misfolded proteins

21 January 2018

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Misfolded proteins partition between two distinct quality control compartments

21 January 2018

1 reference

The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol

21 January 2018

1 reference

A novel quality control compartment derived from the endoplasmic reticulum

21 January 2018

1 reference

The role of ubiquitylation in nerve cell development

21 January 2018

1 reference

The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress

21 January 2018

1 reference

Withaferin A induces proteasome inhibition, endoplasmic reticulum stress, the heat shock response and acquisition of thermotolerance

21 January 2018

1 reference

Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice

21 January 2018

1 reference

Molecular chaperone functions in protein folding and proteostasis

21 January 2018

1 reference

An inhibitor of a deubiquitinating enzyme regulates ubiquitin homeostasis

21 January 2018

1 reference

An analytical solution to the kinetics of breakable filament assembly

21 January 2018

1 reference

The amyloid state and its association with protein misfolding diseases.

21 January 2018

1 reference

Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice

21 January 2018

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Aggresomes, inclusion bodies and protein aggregation

21 January 2018

1 reference

Amyloid-beta-stimulated plasminogen activation by tissue-type plasminogen activator results in processing of neuroendocrine factors

21 January 2018

1 reference

The biology of proteostasis in aging and disease

21 January 2018

1 reference

Ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis. Morphology, distribution, and specificity

21 January 2018

1 reference

Proteomic identification of novel proteins in cortical lewy bodies

21 January 2018

1 reference

Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis.

21 January 2018

1 reference

Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis

21 January 2018

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Neuronal intrinsic mechanisms of axon regeneration.

21 January 2018

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Ubiquitin-proteasome system and mitochondria - reciprocity

21 January 2018

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Ubiquitination in postsynaptic function and plasticity

21 January 2018

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Transmission of endoplasmic reticulum stress and pro-inflammation from tumor cells to myeloid cells

21 January 2018

1 reference

TorsinA and heat shock proteins act as molecular chaperones: suppression of alpha-synuclein aggregation

21 January 2018

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Identifying polyglutamine protein species in situ that best predict neurodegeneration

21 January 2018

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Compartment-specific aggregases direct distinct nuclear and cytoplasmic aggregate deposition

21 January 2018

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Spatially organized aggregation of misfolded proteins as cellular stress defense strategy

21 January 2018

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PICALM modulates autophagy activity and tau accumulation

21 January 2018

1 reference

From cartoon to real time MRI: in vivo monitoring of phagocyte migration in mouse brain

21 January 2018

1 reference

The heat shock response: systems biology of proteotoxic stress in aging and disease

21 January 2018

1 reference

17-AAG, an Hsp90 inhibitor, causes kinetochore defects: a novel mechanism by which 17-AAG inhibits cell proliferation

21 January 2018

1 reference

Dynamic changes in the localization of thermally unfolded nuclear proteins associated with chaperone-dependent protection

21 January 2018

1 reference

Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis

21 January 2018

1 reference

Induced pluripotent stem cells as tools for disease modelling and drug discovery in Alzheimer's disease

21 January 2018

1 reference

A platform to view huntingtin exon 1 aggregation flux in the cell reveals divergent influences from chaperones hsp40 and hsp70.

21 January 2018

1 reference

Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron

21 January 2018

1 reference

The unfolded-protein-response sensor IRE-1α regulates the function of CD8α+ dendritic cells.

21 January 2018

1 reference

Delayed development of adult-generated granule cells in dentate gyrus.

21 January 2018

1 reference

Locus coeruleus neurons are resistant to dysfunction and degeneration by maintaining free ubiquitin levels although total ubiquitin levels decrease upon disruption of polyubiquitin gene Ubb

21 January 2018

1 reference

Ubiquitin is part of the retrovirus budding machinery

21 January 2018

1 reference

Over-expression of heat shock factor 1 phenocopies the effect of chronic inhibition of TOR by rapamycin and is sufficient to ameliorate Alzheimer's-like deficits in mice modeling the disease.

21 January 2018

1 reference

Misfolded polyglutamine, polyalanine, and superoxide dismutase 1 aggregate via distinct pathways in the cell

21 January 2018

1 reference

Clusterin is an ATP-independent chaperone with very broad substrate specificity that stabilizes stressed proteins in a folding-competent state

21 January 2018

1 reference

Targeting Hsp90/Hsp70-based protein quality control for treatment of adult onset neurodegenerative diseases

21 January 2018

1 reference

Neuronal autophagy as a mediator of life and death: contrasting roles in chronic neurodegenerative and acute neural disorders

21 January 2018

1 reference

Regulation of mammalian autophagy in physiology and pathophysiology.

21 January 2018

1 reference

TRPV1 pain receptors regulate longevity and metabolism by neuropeptide signaling.

21 January 2018

1 reference

Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype.

21 January 2018

1 reference

Autophagy and its possible roles in nervous system diseases, damage and repair

21 January 2018

1 reference

The mouse polyubiquitin gene Ubb is essential for meiotic progression

21 January 2018

1 reference

A nonfibrin macromolecular cofactor for tPA-mediated plasmin generation following cellular injury

21 January 2018

1 reference

Nucleocytoplasmic coagulation: an injury-induced aggregation event that disulfide crosslinks proteins and facilitates their removal by plasmin

21 January 2018

1 reference

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

21 January 2018

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Proteasome activator enhances survival of Huntington's disease neuronal model cells

21 January 2018

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Cell type- and brain region-resolved mouse brain proteome.

21 January 2018

1 reference

Celastrol, an oral heat shock activator, ameliorates multiple animal disease models of cell death

21 January 2018

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The HSP70 molecular chaperone is not beneficial in a mouse model of alpha-synucleinopathy

21 January 2018

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Neuronal GPCR controls innate immunity by regulating noncanonical unfolded protein response genes

21 January 2018

1 reference

ALIS are stress-induced protein storage compartments for substrates of the proteasome and autophagy

21 January 2018

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p53 accumulation due to down-regulation of ubiquitin: relevance for neuronal apoptosis

21 January 2018

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Downregulation of free ubiquitin: a novel mechanism of p53 stabilization and neuronal cell death

21 January 2018

1 reference

Neuronal serotonin release triggers the heat shock response in C. elegans in the absence of temperature increase

21 January 2018

1 reference

XBP-1 is a cell-nonautonomous regulator of stress resistance and longevity

21 January 2018

1 reference

Protein aggregation and defective RNA metabolism as mechanisms for motor neuron damage

21 January 2018

1 reference

Small heat-shock proteins: important players in regulating cellular proteostasis

21 January 2018

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Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration.

21 January 2018

1 reference

Effect of αB-crystallin on protein aggregation in Drosophila.

21 January 2018

1 reference

The interplay of neuronal mitochondrial dynamics and bioenergetics: implications for Parkinson's disease

21 January 2018

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Chaperone networks: tipping the balance in protein folding diseases

21 January 2018

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Widespread Proteome Remodeling and Aggregation in Aging C. elegans

21 January 2018

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Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.

21 January 2018

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The interaction of alphaB-crystallin with mature alpha-synuclein amyloid fibrils inhibits their elongation

21 January 2018

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Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity

21 January 2018

1 reference

Exploring the link between glucocerebrosidase mutations and parkinsonism

21 January 2018

1 reference

Small heat shock protein HspB8: its distribution in Alzheimer's disease brains and its inhibition of amyloid-beta protein aggregation and cerebrovascular amyloid-beta toxicity

21 January 2018

1 reference

Clusterin facilitates in vivo clearance of extracellular misfolded proteins

21 January 2018

1 reference

Extracellular chaperones and proteostasis.

21 January 2018

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Hypochlorite-induced structural modifications enhance the chaperone activity of human α2-macroglobulin

21 January 2018

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Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin

21 January 2018

1 reference

Boosting chaperone-mediated autophagy in vivo mitigates α-synuclein-induced neurodegeneration

21 January 2018

1 reference

Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation

21 January 2018

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Protein aggregation can inhibit clathrin-mediated endocytosis by chaperone competition

21 January 2018

1 reference

Proteins that bind to misfolded mutant superoxide dismutase-1 in spinal cords from transgenic amyotrophic lateral sclerosis (ALS) model mice

21 January 2018

1 reference

Clusterin Has Chaperone-like Activity Similar to That of Small Heat Shock Proteins

21 January 2018

1 reference

Alteration of familial ALS-linked mutant SOD1 solubility with disease progression: Its modulation by the proteasome and Hsp70

21 January 2018

1 reference

Increased plasmin-α2-antiplasmin levels indicate activation of the fibrinolytic system in systemic amyloidoses

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1111/JNC.13575

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5 September 2017

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