(Q35095142)

6 August 2017

1 reference

title

Targeting aggregation in the development of therapeutics for the treatment of Huntington's disease and other polyglutamine repeat diseases (English)

1 reference

6 August 2017

0 references

Joan S Steffan

1

1 reference

6 August 2017

Leslie Michels Thompson

series ordinal

2

1 reference

6 August 2017

language of work or name

English

0 references

publication date

1 April 2003

1 reference

Expert Opinion on Therapeutic Targets

6 August 2017

published in

1 reference

6 August 2017

volume

7

1 reference

6 August 2017

issue

2

1 reference

6 August 2017

page(s)

201-213

1 reference

Glutamine repeats and neurodegeneration

6 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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/10.1517%2F14728222.7.2.201

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

CAG repeat number governs the development rate of pathology in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Protein aggregation and pathogenesis of Huntington's disease: mechanisms and correlations

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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/10.1517%2F14728222.7.2.201

Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Transcriptional dysregulation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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/10.1517%2F14728222.7.2.201

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/10.1517%2F14728222.7.2.201

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Microarrays and polyglutamine disorders: reports from the Hereditary Disease Array Group

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Genetic suppression of polyglutamine toxicity in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Altered transcription in yeast expressing expanded polyglutamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Histone deacetylase inhibitors reduce polyglutamine toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Histone deacetylase inhibitor selectively induces p21WAF1 expression and gene-associated histone acetylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Bioenergetics in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Therapeutic opportunities in polyglutamine disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

A microtiter plate assay for polyglutamine aggregate extension

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Human single-chain Fv intrabodies counteract in situ huntingtin aggregation in cellular models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Sequence-specific cleavage of Huntingtin mRNA by catalytic DNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Inhibition of Huntington synthesis by antisense oligodeoxynucleotides

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

siRNA-mediated gene silencing in vitro and in vivo.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Role of the heat shock response and molecular chaperones in oncogenesis and cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Protein misfolding, amyloid formation, and neurodegeneration: a critical role for molecular chaperones?

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Heat shock proteins: endogenous modulators of apoptotic cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Analysis of heat shock transcription factor for suppression of polyglutamine toxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

The length of polyglutamine tract, its level of expression, the rate of degradation, and the transglutaminase activity influence the formation of intracellular aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Protein aggregates and dementia: is there a common toxicity?

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Toxic proteins in neurodegenerative disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14728222.7.2.201

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/10.1517%2F14728222.7.2.201

21 January 2018

Identifiers

DOI

10.1517/14728222.7.2.201

1 reference

6 August 2017

1 reference