(Q45349098)

13 December 2017

title

Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology (English)

1 reference

13 December 2017

1 reference

Isabel Onofre

3

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Clévio Nóbrega

Clévio Nóbrega

1

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David Albuquerque

David Albuquerque

4

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Luis Pereira De Almeida

Luís Pereira de Almeida

7

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Nicole Déglon

Nicole Déglon

6

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author name string

Isabel Nascimento-Ferreira

2

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13 December 2017

Mariana Conceição

5

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13 December 2017

publication date

1 August 2013

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13 December 2017

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13 December 2017

volume

12

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13 December 2017

issue

4

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13 December 2017

page(s)

441-455

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Trinucleotide Repeat Disorders

13 December 2017

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Improvement in the molecular diagnosis of Machado-Joseph disease

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

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

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Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3).

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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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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

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In vivo transduction of murine cerebellar Purkinje cells by HIV-derived lentiviral vectors

21 January 2018

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Exposure of lentiviral vectors to subneutral pH shifts the tropism from Purkinje cell to Bergmann glia

21 January 2018

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21 January 2018

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21 January 2018

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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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21 January 2018

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High transgene expression by lentiviral vectors causes maldevelopment of Purkinje cells in vivo

21 January 2018

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21 January 2018

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21 January 2018

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Intranuclear inclusions, neuronal loss and CAG mosaicism in two patients with Machado-Joseph disease.

21 January 2018

1 reference

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Degeneration of the external cuneate nucleus in spinocerebellar ataxia type 3 (Machado-Joseph disease).

21 January 2018

1 reference

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Spinocerebellar ataxia type 3 (Machado-Joseph disease): severe destruction of the lateral reticular nucleus

21 January 2018

1 reference

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Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation.

21 January 2018

1 reference

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Patterned Purkinje cell death in the cerebellum

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease.

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Behavioral problems as predictors of functional abilities of vascular dementia patients

21 January 2018

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12 December 2020

Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3

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12 December 2020

Genetic correlations in validating emotionality.

1 reference

12 December 2020

Machado-Joseph disease: cerebellar ataxia and autonomic dysfunction in a patient with the shortest known expanded allele (56 CAG repeat units) of the MJD1 gene

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12 December 2020

Using knockout and transgenic mice to study neurophysiology and behavior.

1 reference

12 December 2020

Characterization of mutant mice that express polyglutamine in cerebellar Purkinje cells

1 reference

12 December 2020

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.

1 reference

12 December 2020

Neuropathology and neurodegeneration in rodent brain induced by lentiviral vector-mediated overexpression of alpha-synuclein

1 reference

12 December 2020

Machado-Joseph disease.

1 reference

12 December 2020

10.1007/S12311-012-0432-0

Identifiers

DOI

1 reference

Dimensions Publication ID

13 December 2017

1028839288

0 references

1 reference