Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3. (Q37423625)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19783548%20AND%20SRC:MED&resulttype=core&format=json

8 February 2020

title

Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3 (English)

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8 February 2020

0 references

4

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8 February 2020

author name string

Joonil Jung

series ordinal

1

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8 February 2020

Kexiang Xu

series ordinal

2

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8 February 2020

Derek Lessing

series ordinal

3

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8 February 2020

publication date

25 September 2009

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8 February 2020

published in

Human Molecular Genetics

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8 February 2020

volume

18

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8 February 2020

issue

24

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8 February 2020

page(s)

4843-4852

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Caspases as therapeutic targets in Alzheimer's disease: is it time to "cut" to the chase?

8 February 2020

cites work

1 reference

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Identification of chemical inhibitors to human tissue transglutaminase by screening existing drug libraries

23 August 2017

1 reference

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RNA toxicity is a component of ataxin-3 degeneration in Drosophila

23 August 2017

1 reference

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Study of subcellular localization and proteolysis of ataxin-3

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila

23 August 2017

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Gamma-secretase modulation and its promise for Alzheimer's disease: a rationale for drug discovery.

23 August 2017

1 reference

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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

23 August 2017

1 reference

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An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis

23 August 2017

1 reference

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The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation

23 August 2017

1 reference

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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

23 August 2017

1 reference

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Caspase cleavage of tau: linking amyloid and neurofibrillary tangles in Alzheimer's disease

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

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.

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Suppression of polyglutamine toxicity by the yeast Sup35 prion domain in Drosophila

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

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

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Small N-terminal mutant huntingtin fragments, but not wild type, are mainly present in monomeric form: Implications for pathogenesis

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

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

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Caspase-cleaved tau accumulation in neurodegenerative diseases associated with tau and alpha-synuclein pathology.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

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

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

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

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

Tissue transglutaminase selectively modifies proteins associated with truncated mutant huntingtin in intact cells

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2778376

2 September 2018

Identifiers

DOI

10.1093/HMG/DDP456

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19783548%20AND%20SRC:MED&resulttype=core&format=json

8 February 2020

1 reference

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8 February 2020

PubMed publication ID

19783548

1 reference