(Q35145866)

6 August 2017

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title

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

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ubiquitin-proteasome system

6 August 2017

main subject

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Sarah J Shoesmith Berke

series ordinal

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6 August 2017

Henry L Paulson

series ordinal

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6 August 2017

publication date

1 June 2003

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Current Opinion in Genetics & Development

6 August 2017

published in

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6 August 2017

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6 August 2017

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6 August 2017

page(s)

253-261

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Chaperoning brain degeneration

6 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Parkin protects against the toxicity associated with mutant alpha-synuclein: proteasome dysfunction selectively affects catecholaminergic neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Inhibition of the ubiquitin-proteasome system in Alzheimer's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Inducible expression of mutant alpha-synuclein decreases proteasome activity and increases sensitivity to mitochondria-dependent apoptosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The ubiquitin-proteasome proteolytic pathway: destruction for the sake of construction

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Themes and variations on ubiquitylation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The base of the proteasome regulatory particle exhibits chaperone-like activity

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Role of Rpn11 metalloprotease in deubiquitination and degradation by the 26S proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

A cryptic protease couples deubiquitination and degradation by the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Ubiquitylation of BAG-1 suggests a novel regulatory mechanism during the sorting of chaperone substrates to the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Protein regulation by monoubiquitin

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Regulation of transcriptional activation domain function by ubiquitin

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

How the ubiquitin-proteasome system controls transcription

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

CHIP is associated with Parkin, a gene responsible for familial Parkinson's disease, and enhances its ubiquitin ligase activity

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The ubiquitin pathway in Parkinson's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The DNA repair protein rad23 is a negative regulator of multi-ubiquitin chain assembly

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Endoplasmic reticulum stress and the unfolded protein response in cellular models of Parkinson's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Aggresomes, inclusion bodies and protein aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Parkin accumulation in aggresomes due to proteasome impairment

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Aggresomes: a cellular response to misfolded proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Polyglutamine protein aggregates are dynamic

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Polyglutamine fibrillogenesis: the pathway unfolds

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Arfaptin 2 regulates the aggregation of mutant huntingtin protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

alpha-synuclein metabolism and aggregation is linked to ubiquitin-independent degradation by the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Proteasomal inhibition leads to formation of ubiquitin/alpha-synuclein-immunoreactive inclusions in PC12 cells

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Degradation of alpha-synuclein by proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Spongiform degeneration in mahoganoid mutant mice

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Impaired proteasome function in Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Proteasomal function is impaired in substantia nigra in Parkinson's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The proteasome in brain aging

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Impairment of proteasome structure and function in aging.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Ubiquitin-activating/conjugating activity of TAFII250, a mediator of activation of gene expression in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Protein misfolding, amyloid formation, and neurodegeneration: a critical role for molecular chaperones?

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Harnessing the ubiquitination machinery to target the degradation of specific cellular proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

Gene silencing in mammals by small interfering RNAs

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

siRNA-mediated gene silencing in vitro and in vivo.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-437X%2803%2900053-4

7 January 2021

10.1016/S0959-437X(03)00053-4

Identifiers

DOI

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6 August 2017

1 reference