(Q39995612)

16 September 2017

title

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

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16 September 2017

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Ana Luísa Carvalho

7

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Luis Pereira De Almeida

Luís Pereira de Almeida

13

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Emmanuel Brouillet

Emmanuel Brouillet

10

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Maria C Pedroso de Lima

Maria C Pedroso de Lima

9

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Nicole Déglon

Nicole Déglon

12

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Raymonde Hassig

4

Raymonde Hassig

1 reference

16 September 2017

1

Sandro Alves

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Isabel Nascimento-Ferreira

16 September 2017

3

Isabel Nascimento-Ferreira

1 reference

16 September 2017

2

Etienne Régulier

1 reference

16 September 2017

Noelle Dufour

5

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16 September 2017

Arnulf Koeppen

6

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16 September 2017

Sérgio Simões

8

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16 September 2017

Veronica Colomer Gould

11

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16 September 2017

language of work or name

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1 April 2008

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16 September 2017

published in

Human Molecular Genetics

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16 September 2017

volume

17

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16 September 2017

issue

14

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16 September 2017

page(s)

2071-2083

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

16 September 2017

cites work

1 reference

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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

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

21 January 2018

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Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

21 January 2018

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

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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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

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Pathways to motor incoordination: the inherited ataxias

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Frequency analysis and clinical characterization of spinocerebellar ataxia types 1, 2, 3, 6, and 7 in Korean patients.

21 January 2018

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Dementia and delirium in 4 patients with Machado-Joseph disease

21 January 2018

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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

21 January 2018

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YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.

21 January 2018

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

21 January 2018

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Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence

21 January 2018

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alpha -Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease.

21 January 2018

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Nigrostriatal alpha-synucleinopathy induced by viral vector-mediated overexpression of human alpha-synuclein: a new primate model of Parkinson's disease

21 January 2018

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

21 January 2018

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Polyglutamine disease and neuronal cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Machado-Joseph disease: clinical, molecular, and metabolic characterization in Chinese kindreds.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

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%2FDDN106

Heterogeneous intracellular localization and expression of ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

21 January 2018

1 reference

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

21 January 2018

1 reference

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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

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Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

1 reference

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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%2FHMG%2FDDN106

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Expansion of polyglutamine induces the formation of quasi-aggregate in the early stage of protein fibrillization

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Towards a structural understanding of the fibrillization pathway in Machado-Joseph's disease: trapping early oligomers of non-expanded ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

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%2FDDN106

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%2FDDN106

Molecular clearance of ataxin-3 is regulated by a mammalian E4

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

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%2FDDN106

Autosomal dominant cerebellar ataxia type I. MRI-based volumetry of posterior fossa structures and basal ganglia in spinocerebellar ataxia types 1, 2 and 3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Dystonia and chorea in acquired systemic disorders

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Fluoro-Jade B: a high affinity fluorescent marker for the localization of neuronal degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

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%2FDDN106

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

Machado–Joseph disease in three Scandinavian families

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN106

21 January 2018

Identifiers

DOI

10.1093/HMG/DDN106

1 reference

16 September 2017

1 reference