(Q36067065)

12 August 2017

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title

A novel therapeutic strategy for polyglutamine diseases by stabilizing aggregation-prone proteins with small molecules (English)

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12 August 2017

Motomasa Tanaka

1

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12 August 2017

Yoko Machida

series ordinal

2

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12 August 2017

Nobuyuki Nukina

series ordinal

3

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12 August 2017

publication date

10 March 2005

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Journal of Molecular Medicine

12 August 2017

published in

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12 August 2017

volume

83

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12 August 2017

issue

5

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12 August 2017

page(s)

343-352

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https://scigraph.springernature.com/pub.10.1007/s00109-004-0632-2

12 August 2017

exact match

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cites work

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

1 reference

12 December 2020

Multiple effects of trehalose on protein folding in vitro and in vivo

1 reference

12 December 2020

Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

1 reference

12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Trans-suppression of misfolding in an amyloid disease.

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12 December 2020

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

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12 December 2020

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

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12 December 2020

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

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12 December 2020

Protein folding and misfolding

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12 December 2020

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

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12 December 2020

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

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12 December 2020

Diabetes mellitus in Huntington disease

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12 December 2020

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

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12 December 2020

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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12 December 2020

Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

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12 December 2020

Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease

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12 December 2020

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

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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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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12 December 2020

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

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12 December 2020

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

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12 December 2020

Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease.

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12 December 2020

An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.

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12 December 2020

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

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12 December 2020

Therapeutic approaches to protein-misfolding diseases

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12 December 2020

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

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12 December 2020

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

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12 December 2020

Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila

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12 December 2020

Intra- and intermolecular beta-pleated sheet formation in glutamine-repeat inserted myoglobin as a model for polyglutamine diseases

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12 December 2020

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

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12 December 2020

Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

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12 December 2020

Protein aggregation and neurodegenerative disease

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12 December 2020

Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

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12 December 2020

Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease

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12 December 2020

Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions.

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12 December 2020

Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

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12 December 2020

Oligomeric and polymeric aggregates formed by proteins containing expanded polyglutamine

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12 December 2020

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

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12 December 2020

Prevention of transthyretin amyloid disease by changing protein misfolding energetics.

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12 December 2020

Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease

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12 December 2020

Calpain activation in Huntington's disease.

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12 December 2020

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

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12 December 2020

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

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12 December 2020

Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution

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12 December 2020

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

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12 December 2020

Expansion of polyglutamine induces the formation of quasi-aggregate in the early stage of protein fibrillization

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12 December 2020

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

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12 December 2020

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

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12 December 2020

The effects of aggregation-inducing motifs on amyloid formation of model proteins related to neurodegenerative diseases.

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12 December 2020

Pathogenesis of polyglutamine disorders: aggregation revisited

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12 December 2020

Trehalose expression confers desiccation tolerance on human cells

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12 December 2020

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

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12 December 2020

Polyglutamine fibrillogenesis: the pathway unfolds

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12 December 2020

Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes

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12 December 2020

A cellular model that recapitulates major pathogenic steps of Huntington's disease

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12 December 2020

Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

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12 December 2020

A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila

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12 December 2020

Chronic lithium chloride treatment has variable effects on motor behaviour and survival of mice transgenic for the Huntington's disease mutation

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12 December 2020

Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

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12 December 2020

Huntingtin aggregation and toxicity in Huntington's disease

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12 December 2020

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

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12 December 2020

Solubilization and disaggregation of polyglutamine peptides

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12 December 2020

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

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12 December 2020

Geldanamycin induces Hsp70 and prevents alpha-synuclein aggregation and toxicity in vitro

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12 December 2020

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

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12 December 2020

Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

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12 December 2020

Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

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12 December 2020

Minocycline and doxycycline are not beneficial in a model of Huntington's disease

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12 December 2020

Therapeutic effects of cystamine in a murine model of Huntington's disease.

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12 December 2020

Therapeutic opportunities in polyglutamine disease

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12 December 2020

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

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12 December 2020

Identification of ubiquitin-interacting proteins in purified polyglutamine aggregates

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12 December 2020

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

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12 December 2020

Peptide models for inherited neurodegenerative disorders: conformation and aggregation properties of long polyglutamine peptides with and without interruptions

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12 December 2020

Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells.

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12 December 2020

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

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12 December 2020

Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders

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12 December 2020

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

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12 December 2020

Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.

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12 December 2020

Lipoic acid improves survival in transgenic mouse models of Huntington's disease

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12 December 2020

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

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12 December 2020

Pharmacological prevention of Parkinson disease in Drosophila.

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12 December 2020

Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

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12 December 2020

Inhibiting transthyretin amyloid fibril formation via protein stabilization

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12 December 2020

A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor.

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12 December 2020

Polyglutamine expansion in ataxin-3 does not affect protein stability: implications for misfolding and disease.

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12 December 2020

Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach

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12 December 2020

Polyglutamine and CBP: fatal attraction?

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12 December 2020

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

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12 December 2020

10.1007/S00109-004-0632-2

Identifiers

DOI

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12 August 2017

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