(Q28287762)

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Siddhartha Mitra

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Erik S Schweitzer

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Mark R Segal

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Steven Finkbeiner

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

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language of work or name

English

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publication date

14 October 2004

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published in

Nature

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volume

431

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issue

7010

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page(s)

805-10

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cites work

1 reference

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

21 April 2017

1 reference

A monomeric red fluorescent protein

21 April 2017

1 reference

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

21 April 2017

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Impairment of the ubiquitin-proteasome system by protein aggregation

21 April 2017

1 reference

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Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

21 April 2017

1 reference

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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/10.1038%2FNATURE02998

7 January 2021

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Expanded polyglutamine stretches form an 'aggresome'.

1 reference

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7 January 2021

Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

1 reference

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7 January 2021

Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

1 reference

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7 January 2021

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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/10.1038%2FNATURE02998

7 January 2021

Green fluorescent protein as a novel tool to measure apoptosis and necrosis

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Molecular genetics of Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

A one-hit model of cell death in inherited neuronal degenerations

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Differential loss of striatal projection neurons in Huntington disease

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Specificity in intracellular protein aggregation and inclusion body formation

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Evidence for a recruitment and sequestration mechanism in Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Green fluorescent protein as a quantitative tool

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Differential expression of normal and mutant Huntington's disease gene alleles

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Nuclear targeting of mutant Huntingtin increases toxicity

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

CREB: a major mediator of neuronal neurotrophin responses

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021

A cell-based screen for drugs to treat Huntington's disease

1 reference

https://api.crossref.org/works/10.1038%2FNATURE02998

7 January 2021