(Q33374523)

26 July 2017

Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease (English)

1 reference

26 July 2017

Machado-Joseph disease

1 reference

inferred from title

author

Luis Pereira De Almeida

Luís Pereira de Almeida

9

0 references

Emmanuel Brouillet

Emmanuel Brouillet

6

0 references

Maria C Pedroso de Lima

Maria C Pedroso de Lima

7

0 references

Nicole Déglon

Nicole Déglon

10

0 references

Philippe Hantraye

8

Philippe Hantraye

1 reference

26 July 2017

3

Gwennaëlle Auregan

1 reference

26 July 2017

4

Raymonde Hassig

1 reference

26 July 2017

1

Sandro Alves

1 reference

PubMed ID

18841197

Isabel Nascimento-Ferreira

26 July 2017

2

Isabel Nascimento-Ferreira

1 reference

26 July 2017

Noëlle Dufour

5

1 reference

PubMed ID

18841197

26 July 2017

language of work or name

English

0 references

publication date

8 October 2008

1 reference

26 July 2017

1 reference

26 July 2017

3

1 reference

26 July 2017

10

1 reference

26 July 2017

e3341

1 reference

Creative Commons Attribution 4.0 International

26 July 2017

start time

8 October 2008

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

cites work

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

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

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

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

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

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

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Rational siRNA design for RNA interference

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Poly-ubiquitin Binding by the Polyglutamine Disease Protein Ataxin-3 Links Its Normal Function to Protein Surveillance Pathways

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Allele-specific silencing of dominant disease genes

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Spinocerebellar ataxia type 3 phenotypically resembling parkinson disease in a black family

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Complete and long-term rescue of lesioned adult motoneurons by lentiviral-mediated expression of glial cell line-derived neurotrophic factor in the facial nucleus.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Homozygosity for Machado-Joseph disease gene enhances phenotypic severity

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

14 July 2018

Machado-Joseph disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Marked phenotypic heterogeneity associated with expansion of a CAG repeat sequence at the spinocerebellar ataxia 3/Machado-Joseph disease locus

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Dopamine transporter positron emission tomography in spinocerebellar ataxias type 1, 2, 3, and 6.

14 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Allele-specific RNAi selectively silences mutant SOD1 and achieves significant therapeutic benefit in vivo.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Linkage disequilibrium analysis in Machado-Joseph disease patients of different ethnic origins

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Homozygous inheritance of the Machado-Joseph disease gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Linkage disequilibrium at the Machado-Joseph disease/spinal cerebellar ataxia 3 locus: evidence for a common founder effect in French and Portuguese-Brazilian families as well as a second ancestral Portuguese-Azorean mutation.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2553199

Molecular features of the CAG repeats and clinical manifestation of Machado--Joseph disease

31 October 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0003341

Toward therapy for DYT1 dystonia: allele-specific silencing of mutant TorsinA

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/18841197

12 December 2020

inferred from PubMed ID database lookup

How inaccurate is the Abercrombie correction factor for cell counts?

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/18841197

12 December 2020

inferred from PubMed ID database lookup

Relationship of (CAG)nC configuration to repeat instability of the Machado-Joseph disease gene

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/18841197

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1371/JOURNAL.PONE.0003341

1 reference

26 July 2017

0 references

1 reference

26 July 2017

1 reference

PubMed ID

18841197