(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

12

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author name string

Caroline L Benn

1

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11 December 2017

Christian Landles

2

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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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11 December 2017

3065-3078

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11 December 2017

20

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

11 December 2017

1 reference

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

1 reference

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

1 reference

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

Orexin loss in Huntington's disease.

21 January 2018

1 reference

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

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

21 January 2018

1 reference

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

1 reference

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

1 reference

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

1 reference

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

1 reference

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

1 reference

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

1 reference

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

1 reference

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

A short amino acid sequence able to specify nuclear location

21 January 2018

1 reference

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

1 reference

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

1 reference

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

1 reference

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

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

21 January 2018

1 reference

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

1 reference

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

1 reference

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

1 reference

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

Polyglutamine expansion of huntingtin impairs its nuclear export

21 January 2018

1 reference

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

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

21 January 2018

1 reference

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

1 reference

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

1 reference

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

Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

21 January 2018

1 reference

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

1 reference

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

1 reference

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

Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release

21 January 2018

1 reference

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

1 reference

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

Deranged neuronal calcium signaling and Huntington disease.

21 January 2018

1 reference

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

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

21 January 2018

1 reference

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

1 reference

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

Neurotransmitter receptor analysis in transgenic mouse models

21 January 2018

1 reference

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

1 reference

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

1 reference

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

1 reference

11 December 2017

1 reference