(Q48373570)

11 February 2018

title

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

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11 February 2018

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11 February 2018

Huda Zoghbi

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11 February 2018

Harry T. Orr

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11 February 2018

author name string

Klement IA

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11 February 2018

Kaytor MD

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11 February 2018

Yi H

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11 February 2018

Hersch SM

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11 February 2018

Clark HB

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11 February 2018

language of work or name

English

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publication date

1 October 1998

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11 February 2018

published in

Cell

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11 February 2018

volume

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11 February 2018

issue

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11 February 2018

page(s)

41-53

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11 February 2018

Dentatorubral and pallidoluysian atrophy (DRPLA) Clinical and neuropathological findings in genetically confirmed north american and european pedigrees

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

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

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

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Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

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

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Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

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Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

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Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase

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Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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7 January 2021

The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1

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7 January 2021

Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p

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Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse

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Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.

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Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

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Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.

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The intranuclear location of a herpes simplex virus DNA-binding protein is determined by the status of viral DNA replication

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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

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT.

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7 January 2021

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

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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

Widespread expression of Huntington's disease gene (IT15) protein product

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7 January 2021

A nuclear localization domain in the hnRNP A1 protein

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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

HIP-I: a huntingtin interacting protein isolated by the yeast two-hybrid system

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7 January 2021

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

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7 January 2021

Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

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7 January 2021

10.1016/S0092-8674(00)81781-X

Identifiers

DOI

1 reference

11 February 2018

1 reference

11 February 2018