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

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A monomeric red fluorescent protein

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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

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

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

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

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

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

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

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Expanded polyglutamine stretches form an 'aggresome'.

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

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

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Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

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Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

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

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

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

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

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

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

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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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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.

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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

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

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Molecular genetics of Huntington's disease

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

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Differential loss of striatal projection neurons in Huntington disease

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

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Specificity in intracellular protein aggregation and inclusion body formation

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

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

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

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Green fluorescent protein as a quantitative tool

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

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

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

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Nuclear targeting of mutant Huntingtin increases toxicity

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Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

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

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

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CREB: a major mediator of neuronal neurotrophin responses

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

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