(Q44437509)

3 December 2017

title

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice (English)

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3 December 2017

Harry T Orr

8

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Huda Zoghbi

object named as

Huda Y Zoghbi

6

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author name string

Effat S Emamian

1

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3 December 2017

Michael D Kaytor

2

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3 December 2017

Lisa A Duvick

3

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3 December 2017

Tao Zu

4

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3 December 2017

Susan K Tousey

5

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3 December 2017

H Brent Clark

7

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3 December 2017

language of work or name

English

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publication date

1 May 2003

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3 December 2017

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3 December 2017

volume

38

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3 December 2017

issue

3

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3 December 2017

page(s)

375-387

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Identification and characterization of the gene causing type 1 spinocerebellar ataxia

3 December 2017

cites work

1 reference

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SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

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7 January 2021

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

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7 January 2021

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

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7 January 2021

Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.

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7 January 2021

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

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7 January 2021

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

1 reference

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7 January 2021

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

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Identification of genes that modify ataxin-1-induced neurodegeneration.

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Clinical, neuropathologic, and genetic studies of a large spinocerebellar ataxia type 1 (SCA1) kindred

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Signaling--2000 and beyond

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Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2.

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Abundant expression and cytoplasmic aggregations of [alpha]1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6

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Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait

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Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout?

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7 January 2021

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

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7 January 2021

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

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7 January 2021

SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein

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7 January 2021

Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death

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7 January 2021

Beyond the Qs in the polyglutamine diseases

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7 January 2021

Structural and immunocytochemical features of olivopontocerebellar atrophy caused by the spinocerebellar ataxia type 1 (SCA-1) mutation define a unique phenotype

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7 January 2021

Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

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7 January 2021

Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals

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7 January 2021

Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

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7 January 2021

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

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7 January 2021

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

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7 January 2021

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

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7 January 2021

PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival

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7 January 2021

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

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7 January 2021

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7 January 2021

The expanding world of ataxins

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7 January 2021

Mouse and fly models of neurodegeneration

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7 January 2021

Glutamine repeats and neurodegeneration

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7 January 2021

10.1016/S0896-6273(03)00258-7

Identifiers

DOI

1 reference

3 December 2017

1 reference