Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease (Q28568604)

Isabel Onofre

5

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Tiago Santos-Ferreira

Tiago Santos-Ferreira

2

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Luis Pereira De Almeida

Luís Pereira de Almeida

11

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Nicole Déglon

Nicole Déglon

10

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author name string

Isabel Nascimento-Ferreira

1

1 reference

31 January 2017

Gwennaëlle Auregan

4

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31 January 2017

Sandro Alves

6

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31 January 2017

Noëlle Dufour

7

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31 January 2017

Veronica F. Colomer Gould

8

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31 January 2017

Arnulf Koeppen

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

9

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31 January 2017

language of work or name

English

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publication date

1 May 2011

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31 January 2017

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31 January 2017

134

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31 January 2017

1400–1415

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31 January 2017

Pt 5

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31 January 2017

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWR047

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

21 January 2018

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The dynamics of autophagy visualized in live cells: from autophagosome formation to fusion with endo/lysosomes

21 January 2018

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p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death

21 January 2018

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Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease

21 January 2018

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Physiological functions of Atg6/Beclin 1: a unique autophagy-related protein

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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Selective molecular alterations in the autophagy pathway in patients with Lewy body disease and in models of alpha-synucleinopathy

21 January 2018

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Autophagy: in sickness and in health

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Self-inactivating lentiviral vectors with enhanced transgene expression as potential gene transfer system in Parkinson's disease

21 January 2018

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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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Molecular pathogenesis of spinocerebellar ataxias.

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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Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

21 January 2018

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Selective degradation of p62 by autophagy

21 January 2018

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Regulation of amyloid precursor protein processing by the Beclin 1 complex

21 January 2018

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Autophagy genes protect against disease caused by polyglutamine expansion proteins in Caenorhabditis elegans

21 January 2018

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LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing

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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Rapamycin inhibits polyglutamine aggregation independently of autophagy by reducing protein synthesis

21 January 2018

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A role for ubiquitin in selective autophagy

21 January 2018

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Loss of autophagy in the central nervous system causes neurodegeneration in mice

21 January 2018

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Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice

21 January 2018

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Intracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseases

21 January 2018

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Induction of autophagy and inhibition of tumorigenesis by beclin 1

21 January 2018

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Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3

21 January 2018

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Methods in mammalian autophagy research

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Extensive involvement of autophagy in Alzheimer disease: an immuno-electron microscopy study.

21 January 2018

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p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

21 January 2018

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The roles of intracellular protein-degradation pathways in neurodegeneration

21 January 2018

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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

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Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1

21 January 2018

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Utilizing flow cytometry to monitor autophagy in living mammalian cells

21 January 2018

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Promoting basal levels of autophagy in the nervous system enhances longevity and oxidant resistance in adult Drosophila

21 January 2018

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Beclin 1 gene transfer activates autophagy and ameliorates the neurodegenerative pathology in alpha-synuclein models of Parkinson's and Lewy body diseases

21 January 2018

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Inactivation of the autophagy gene bec-1 triggers apoptotic cell death in C. elegans

21 January 2018

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Autophagy genes and ageing

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Aggregate-prone proteins are cleared from the cytosol by autophagy: therapeutic implications

21 January 2018

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Autophagy gone awry in neurodegenerative diseases

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TOR signaling in growth and metabolism

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Autophagy: molecular machinery for self-eating

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Macroautophagy--a novel Beta-amyloid peptide-generating pathway activated in Alzheimer's disease

21 January 2018

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Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment

21 January 2018

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Functional specificity of the mammalian Beclin-Vps34 PI 3-kinase complex in macroautophagy versus endocytosis and lysosomal enzyme trafficking

21 January 2018

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21 January 2018

Spinocerebellar Ataxias

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