Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going? (Q37735565)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

review article

1 reference

title

Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going? (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

0 references

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

author name string

Toshiaki Takahashi

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

Shinichi Katada

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

publication date

21 April 2010

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

Journal of Molecular Cell Biology

31 January 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

volume

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

issue

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

31 January 2020

page(s)

180-191

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20410236%20AND%20SRC:MED&resulttype=core&format=json

CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model.

31 January 2020

cites work

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Effects of Coenzyme Q10 in Huntington's disease and early Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

DNA repair in neural cells: basic science and clinical implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

The promises and pitfalls of RNA-interference-based therapeutics

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Huntingtin facilitates dynein/dynactin-mediated vesicle transport.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

CHIP activates HSF1 and confers protection against apoptosis and cellular stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Calcium dysregulation and membrane disruption as a ubiquitous neurotoxic mechanism of soluble amyloid oligomers

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Biologically active molecules that reduce polyglutamine aggregation and toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Defective DNA single-strand break repair in spinocerebellar ataxia with axonal neuropathy-1

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Diseases of unstable repeat expansion: mechanisms and common principles

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Live-cell fluorescent biosensors for activated signaling proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Purification of neuronal inclusions of patients with Huntington's disease reveals a broad range of N-terminal fragments of expanded huntingtin and insoluble polymers

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

CREB-binding protein modulates repeat instability in a Drosophila model for polyQ disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Permeabilization of lipid bilayers is a common conformation-dependent activity of soluble amyloid oligomers in protein misfolding diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Genetic suppression of polyglutamine toxicity in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Transvascular delivery of small interfering RNA to the central nervous system.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Trinucleotide repeat expansion in neurological disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

A century-old debate on protein aggregation and neurodegeneration enters the clinic

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine repeats of spinocerebellar ataxia 6 impair the cell-death-preventing effect of CaV2.1 Ca2+ channel--loss-of-function cellular model of SCA6.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine expansion diseases: failing to deliver

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Modulation of neurodegeneration by molecular chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Beyond the Qs in the polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Regulation of cell death: the calcium-apoptosis link

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Amyloid fibers are water-filled nanotubes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Proteome analysis of soluble nuclear proteins reveals that HMGB1/2 suppress genotoxic stress in polyglutamine diseases.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Role and fate of PML nuclear bodies in response to interferon and viral infections

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Protein aggregation and neurodegenerative disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Opinion: What is the role of protein aggregation in neurodegeneration?

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Polyglutamine fibrillogenesis: the pathway unfolds

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Interference of CREB-dependent transcriptional activation by expanded polyglutamine stretches--augmentation of transcriptional activation as a potential therapeutic strategy for polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Evidence that Perutz's double-beta-stranded subunit structure for beta-amyloids also applies to their channel-forming structures in membranes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Interaction of huntingtin fragments with brain membranes--clues to early dysfunction in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Intra- and intermolecular beta-pleated sheet formation in glutamine-repeat inserted myoglobin as a model for polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

SUMO-1 co-localized with mutant atrophin-1 with expanded polyglutamines accelerates intranuclear aggregation and cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Mutational analysis of the structural organization of polyglutamine aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Suppression of Huntington's disease pathology in Drosophila by human single-chain Fv antibodies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Pathology of CAG repeat diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Partitioning of lipid-modified monomeric GFPs into membrane microdomains of live cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FJMCB%2FMJQ005