(Q39724719)

14 September 2017

title

Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3? (English)

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14 September 2017

main subject

Machado-Joseph disease

1 reference

Clévio Nóbrega

6

0 references

Luis Pereira De Almeida

Luís Pereira de Almeida

11

0 references

Emmanuel Brouillet

Emmanuel Brouillet

7

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Maria C Pedroso de Lima

Maria C Pedroso de Lima

9

0 references

Nicole Déglon

Nicole Déglon

10

0 references

Philippe Hantraye

8

Philippe Hantraye

1 reference

14 September 2017

5

Gwennaëlle Auregan

1 reference

14 September 2017

4

Raymonde Hassig

1 reference

14 September 2017

1

Sandro Alves

1 reference

Isabel Nascimento-Ferreira

14 September 2017

2

Isabel Nascimento-Ferreira

1 reference

14 September 2017

Noëlle Dufour

3

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14 September 2017

publication date

22 March 2010

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14 September 2017

published in

Human Molecular Genetics

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14 September 2017

volume

19

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14 September 2017

issue

12

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14 September 2017

page(s)

2380-2394

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Machado-Joseph disease: an autosomal dominant motor system degeneration

14 September 2017

cites work

1 reference

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

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The parkinsonian phenotype of spinocerebellar ataxia type 3 in a Taiwanese family

21 January 2018

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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.

21 January 2018

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

21 January 2018

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21 January 2018

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

21 January 2018

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

21 January 2018

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Molecular and clinical correlations in spinocerebellar ataxia type 3 and Machado-Joseph disease

21 January 2018

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Machado-Joseph disease gene product identified in lymphocytes and brain.

21 January 2018

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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Elucidation of ataxin-3 and ataxin-7 function by integrative bioinformatics

21 January 2018

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

21 January 2018

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Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Ataxin-3 is subject to autolytic cleavage.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B

21 January 2018

1 reference

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Fourteen and counting: unraveling trinucleotide repeat diseases

21 January 2018

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Trinucleotide repeats: mechanisms and pathophysiology

21 January 2018

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Improvement in the molecular diagnosis of Machado-Joseph disease

21 January 2018

1 reference

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

21 January 2018

1 reference

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Heterogeneous intracellular localization and expression of ataxin-3.

21 January 2018

1 reference

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Revealing the world of RNA interference

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Duplexes of 21-nucleotide RNAs mediate RNA interference in cultured mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

rAAV-mediated shRNA ameliorated neuropathology in Huntington disease model mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

AAV vector-mediated RNAi of mutant huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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/10.1093%2FHMG%2FDDQ111

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/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Allele-specific silencing of dominant disease genes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Recognition of the polyubiquitin proteolytic signal

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Recruitment of nonexpanded polyglutamine proteins to intranuclear aggregates in neuronal intranuclear hyaline inclusion disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Preferential recruitment of ataxin-3 independent of expanded polyglutamine: an immunohistochemical study on Marinesco bodies

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Intranuclear aggregation of nonexpanded ataxin-3 in marinesco bodies of the nonhuman primate substantia nigra.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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%2FHMG%2FDDQ111

Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Ataxin-3 is transported into the nucleus and associates with the nuclear matrix

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Characterization of the rat spinocerebellar ataxia type 3 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

A genomic and functional inventory of deubiquitinating enzymes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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/10.1093%2FHMG%2FDDQ111

Identification and allele-specific silencing of the mutant huntingtin allele in Huntington's disease patient-derived fibroblasts.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Dopamine gene therapy for Parkinson's disease in a nonhuman primate without associated dyskinesia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

Fluoro-Jade: a novel fluorochrome for the sensitive and reliable histochemical localization of neuronal degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

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/10.1093%2FHMG%2FDDQ111

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/10.1093%2FHMG%2FDDQ111

Bregma, lambda and the interaural midpoint in stereotaxic surgery with rats of different sex, strain and weight

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ111

21 January 2018

Identifiers

DOI

10.1093/HMG/DDQ111

1 reference

14 September 2017

1 reference