Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease (Q45306894)

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English	Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease	scientific article

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scholarly article

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Europe PubMed Central

PubMed publication ID

11 December 2017

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease (English)

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Europe PubMed Central

PubMed publication ID

11 December 2017

Huntington's disease

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7

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11 December 2017

Paolo Paganetti

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Paolo Paganetti

8

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author name string

Andreas Weiss

1

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Europe PubMed Central

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11 December 2017

Corinna Klein

2

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11 December 2017

Ben Woodman

3

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Europe PubMed Central

PubMed publication ID

11 December 2017

Kirupa Sathasivam

4

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11 December 2017

Miriam Bibel

5

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11 December 2017

Etienne Régulier

6

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11 December 2017

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publication date

6 November 2007

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11 December 2017

number of pages

13

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Journal of Neurochemistry

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11 December 2017

104

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11 December 2017

3

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11 December 2017

846-858

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Europe PubMed Central

PubMed publication ID

11 December 2017

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Huntingtin aggregation and toxicity in Huntington's disease

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Impairment of the ubiquitin-proteasome system by protein aggregation

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Formic acid dissolves aggregates of an N-terminal huntingtin fragment containing an expanded polyglutamine tract: applying to quantification of protein components of the aggregates

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Immortalized young adult neurons from the septal region: generation and characterization

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Neurological abnormalities in a knock-in mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Insoluble TATA-binding protein accumulation in Huntington's disease cortex.

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Diffusion tensor imaging in presymptomatic and early Huntington's disease: Selective white matter pathology and its relationship to clinical measures.

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

A new multiphasic buffer system for sodium dodecyl sulfate-polyacrylamide gel electrophoresis of proteins and peptides with molecular masses 100,000-1000, and their detection with picomolar sensitivity

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2007.05032.X

21 January 2018

Identifiers

10.1111/J.1471-4159.2007.05032.X

1 reference

Europe PubMed Central

PubMed publication ID

11 December 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

11 December 2017

ResearchGate publication ID

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