(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

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

21 January 2018

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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

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21 January 2018

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

21 January 2018

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

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Calpain activity in retinal degeneration.

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

1 reference

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDS449

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12 December 2017

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