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

publication date

1 September 2000

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30 July 2017

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30 July 2017

volume

381

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issue

9-10

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page(s)

937-942

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

30 July 2017

cites work

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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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

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Toward understanding the molecular pathology of Huntington's disease.

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

Identifiers

DOI

10.1515/BC.2000.114

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30 July 2017

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