(Q45310677)

12 December 2017

title

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). (English)

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12 December 2017

main subject

spinocerebellar ataxia

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based on heuristic

inferred from title

author

François Paquet-Durand

10

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12 December 2017

8

Ullrich Wüllner

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12 December 2017

13

Olaf Riess

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12 December 2017

14

Huu Phuc Nguyen

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12 December 2017

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1

Jeannette Hübener

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12 December 2017

author name string

Jonasz Jeremiasz Weber

2

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12 December 2017

Claudia Richter

3

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12 December 2017

Lisa Honold

4

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12 December 2017

Andreas Weiss

5

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12 December 2017

Fabronia Murad

6

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12 December 2017

Peter Breuer

7

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12 December 2017

Peter Bellstedt

9

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12 December 2017

Jiro Takano

11

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12 December 2017

Takaomi C Saido

12

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12 December 2017

publication date

24 October 2012

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12 December 2017

published in

Human Molecular Genetics

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12 December 2017

volume

22

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12 December 2017

issue

3

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12 December 2017

page(s)

508-518

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Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

12 December 2017

cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Glutamine repeats and neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Identification and functional dissection of localization signals within ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Polyglutamine pathogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

21 January 2018

Marc Tessier-Lavigne.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Study of subcellular localization and proteolysis of ataxin-3

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Calcium-bound structure of calpain and its mechanism of inhibition by calpastatin

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Disruption of the mouse mu-calpain gene reveals an essential role in platelet function

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Calpain mediates excitotoxic DNA fragmentation via mitochondrial pathways in adult brains: evidence from calpastatin mutant mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Calpain activity in retinal degeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Photoreceptor rescue and toxicity induced by different calpain inhibitors.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Calpain inhibitors: a survey of compounds reported in the patent and scientific literature

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

Proteolytic cleavage of polyglutamine-expanded ataxin-3 is critical for aggregation and sequestration of non-expanded ataxin-3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS449

21 January 2018

Identifiers

DOI

10.1093/HMG/DDS449

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12 December 2017

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