Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice (Q48115369)

8 February 2018

title

Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice (English)

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8 February 2018

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8 February 2018

Martina Marinello

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Alice Chort

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8 February 2018

Sandro Alves

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8 February 2018

Béatrice Dufresnois

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8 February 2018

Jean-Gabriel Dornbierer

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8 February 2018

Christelle Tesson

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8 February 2018

Morwena Latouche

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8 February 2018

Darren P Baker

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8 February 2018

Martine Barkats

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8 February 2018

Khalid H El Hachimi

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8 February 2018

Merle Ruberg

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8 February 2018

Alexandre Janer

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8 February 2018

Alexis Brice

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8 February 2018

Annie Sittler

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8 February 2018

language of work or name

English

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publication date

21 March 2013

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8 February 2018

published in

Brain

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8 February 2018

volume

136

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8 February 2018

issue

Pt 6

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8 February 2018

page(s)

1732-1745

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8 February 2018

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Molecular and clinical studies in SCA-7 define a broad clinical spectrum and the infantile phenotype

21 January 2018

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Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

21 January 2018

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JAKs, STATs and signal transduction in response to the interferons and other cytokines

21 January 2018

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Interference of Crx-dependent transcription by ataxin-7 involves interaction between the glutamine regions and requires the ataxin-7 carboxy-terminal region for nuclear localization

21 January 2018

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Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Nuclear transit of the intracellular domain of the interferon receptor subunit IFNaR2 requires Stat2 and Irf9.

21 January 2018

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Pharmacology and therapeutic potential of interferons

21 January 2018

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Molecular and clinical study of 18 families with ADCA type II: evidence for genetic heterogeneity and de novo mutation

21 January 2018

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Combination therapies in multiple sclerosis

21 January 2018

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Molecular Cloning of a New Interferon-induced PML Nuclear Body-associated Protein

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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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

21 January 2018

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The genetics of neurodegenerative diseases

21 January 2018

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Disease progression despite early loss of polyglutamine protein expression in SCA7 mouse model.

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Ataxin-7 is a subunit of GCN5 histone acetyltransferase-containing complexes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Transcriptional alterations and chromatin remodeling in polyglutamine diseases.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Improvement of neuropathology and transcriptional deficits in CAG 140 knock-in mice supports a beneficial effect of dietary curcumin in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Therapeutic role of beta-interferons in multiple sclerosis

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Steroid-like signalling by interferons: making sense of specific gene activation by cytokines

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Murine interferon-beta receptor-mediated endocytosis and nuclear membrane binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Clastosome: a subtype of nuclear body enriched in 19S and 20S proteasomes, ubiquitin, and protein substrates of proteasome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Polyglutamine-expanded spinocerebellar ataxia-7 protein disrupts normal SAGA and SLIK histone acetyltransferase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Spinocerebellar ataxia type 7 associated with pigmentary retinal dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Distribution of mouse interferon-beta in normal and brain tumour-bearing mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Protein aggregation and neurodegenerative disease

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Opinion: What is the role of protein aggregation in neurodegeneration?

21 January 2018

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https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Behavioral changes are not directly related to striatal monoamine levels, number of nigral neurons, or dose of parkinsonian toxin MPTP in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Clinical and molecular advances in autosomal dominant cerebellar ataxias: from genotype to phenotype and physiopathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

PML nuclear bodies and neuronal intranuclear inclusion in polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Interaction between neuronal intranuclear inclusions and promyelocytic leukemia protein nuclear and coiled bodies in CAG repeat diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

Distribution of ataxin-7 in normal human brain and retina

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FBRAIN%2FAWT061

21 January 2018

Identifiers

DOI

10.1093/BRAIN/AWT061

1 reference

8 February 2018

1 reference

8 February 2018