Protein aggregation and pathogenesis of Huntington's disease: mechanisms and correlations (Q34081469)

30 July 2017

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title

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

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30 July 2017

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Wanker EE

1

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30 July 2017

1 September 2000

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30 July 2017

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30 July 2017

381

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30 July 2017

9-10

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30 July 2017

937-942

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SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

30 July 2017

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the human Huntington disease transcript

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie?

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

Huntingtin immunoreactivity in the rat neostriatum: differential accumulation in projection and interneurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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/10.1515%2FBC.2000.114

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

Toward understanding the molecular pathology of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

alpha-synuclein fibrillogenesis is nucleation-dependent. Implications for the pathogenesis of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1515%2FBC.2000.114

21 January 2018

Identifiers

DOI

10.1515/BC.2000.114

1 reference

30 July 2017

1 reference

PubMed ID

11076024