Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease (Q45298542)

11 December 2017

Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease (English)

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11 December 2017

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15

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11 December 2017

Paul T Sharpe

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author name string

Caroline L Benn

1

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11 December 2017

Christian Landles

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11 December 2017

He Li

3

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11 December 2017

Andrew D Strand

4

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11 December 2017

Ben Woodman

5

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11 December 2017

Kirupa Sathasivam

6

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11 December 2017

Shi-Hua Li

7

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11 December 2017

Shabnam Ghazi-Noori

8

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11 December 2017

Emma Hockly

9

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11 December 2017

Syed M N N Faruque

10

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11 December 2017

Jang-Ho J Cha

11

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11 December 2017

James M Olson

13

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11 December 2017

Xiao-Jiang Li

14

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11 December 2017

23 September 2005

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11 December 2017

Human Molecular Genetics

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11 December 2017

14

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20

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11 December 2017

3065-3078

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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

11 December 2017

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Orexin loss in Huntington's disease.

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Gene expression in Huntington's disease skeletal muscle: a potential biomarker

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

A short amino acid sequence able to specify nuclear location

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Cytoplasmic localization of mitogen-activated protein kinase kinase directed by its NH2-terminal, leucine-rich short amino acid sequence, which acts as a nuclear export signal

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

A novel regulatory mechanism in the mitogen-activated protein (MAP) kinase cascade. Role of nuclear export signal of MAP kinase kinase.

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Behavioral and functional analysis of mouse phenotype: SHIRPA, a proposed protocol for comprehensive phenotype assessment

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Polyglutamine expansion of huntingtin impairs its nuclear export

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Deranged neuronal calcium signaling and Huntington disease.

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Standardization and statistical approaches to therapeutic trials in the R6/2 mouse

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation.

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Neurotransmitter receptor analysis in transgenic mouse models

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDI340

21 January 2018

Identifiers

DOI

10.1093/HMG/DDI340

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11 December 2017

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