Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases (Q34797845)

4 August 2017

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title

Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases (English)

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4 August 2017

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Ray Truant

1

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4 August 2017

Randy Singh Atwal

2

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4 August 2017

Carly Desmond

3

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4 August 2017

Lise Munsie

4

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4 August 2017

Thu Tran

5

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4 August 2017

language of work or name

English

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publication date

15 July 2008

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4 August 2017

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4 August 2017

275

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4 August 2017

17

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4 August 2017

4252-4262

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

4 August 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Glutamine repeats and inherited neurodegenerative diseases: molecular aspects

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Intracellular localization of homopolymeric amino acid-containing proteins expressed in mammalian cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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/10.1111%2FJ.1742-4658.2008.06561.X

Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Evidence for a recruitment and sequestration mechanism in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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/10.1111%2FJ.1742-4658.2008.06561.X

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Amyloid diseases: abnormal protein aggregation in neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington's disease gene results in subtle behavioral/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Wild-type huntingtin plays a role in brain development and neuronal survival

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

RNA association and nucleocytoplasmic shuttling by ataxin-1

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Depletion of wild-type huntingtin in mouse models of neurologic diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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/10.1111%2FJ.1742-4658.2008.06561.X

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Differential susceptibility of cultured cell lines to aggregate formation and cell death produced by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Mutational analysis of the structural organization of polyglutamine aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Detection of polyglutamine protein oligomers in cells by fluorescence correlation spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Flanking sequences profoundly alter polyglutamine toxicity in yeast

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

In-cell aggregation of a polyglutamine-containing chimera is a multistep process initiated by the flanking sequence

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Critical role of the proline-rich region in Huntingtin for aggregation and cytotoxicity in yeast.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Oligoproline effects on polyglutamine conformation and aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

HYPK, a Huntingtin interacting protein, reduces aggregates and apoptosis induced by N-terminal Huntingtin with 40 glutamines in Neuro2a cells and exhibits chaperone-like activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Huntingtin interacting protein HYPK is intrinsically unstructured

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

A Rapid Cellular FRET Assay of Polyglutamine Aggregation Identifies a Novel Inhibitor

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Emerging evidence for the neuroprotective role of alpha-synuclein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Amyloid beta: the alternate hypothesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

New directions for neurodegenerative disease therapy: using chemical compounds to boost the formation of mutant protein inclusions

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Inhibition of polyglutamine protein aggregation and cell death by novel peptides identified by phage display screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

Amino Acid Sequence Requirements of Peptides That Inhibit Polyglutamine-Protein Aggregation and Cell Death

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06561.X

10.1111/J.1742-4658.2008.06561.X

21 January 2018

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DOI

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4 August 2017

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