Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice (Q44931209)

7 December 2017

title

Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice (English)

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7 December 2017

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13

Neal G Copeland

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12

Nancy A Jenkins

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14

Christopher A Ross

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Gabriele Schilling

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Alena V Savonenko

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Alexandra Klevytska

3

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Johanna L Morton

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Stina M Tucker

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Michelle Poirier

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Alexa Gale

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Ning Chan

8

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Vicky Gonzales

9

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Hilda H Slunt

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Michael L Coonfield

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David R Borchelt

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publication date

9 June 2004

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7 December 2017

published in

Human Molecular Genetics

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volume

13

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issue

15

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page(s)

1599-1610

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

7 December 2017

cites work

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Trinucleotide repeats in neurogenetic disorders

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When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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Huntington's Disease and Dentatorubral‐Pallidoluysian Atrophy: Proteins, Pathogenesis and Pathology

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Decreased neuronal and increased oligodendroglial densities in Huntington's disease caudate nucleus

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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

21 January 2018

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Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

21 January 2018

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Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

21 January 2018

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A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

21 January 2018

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

21 January 2018

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A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

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

21 January 2018

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

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

21 January 2018

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In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

21 January 2018

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Nuclear targeting of mutant Huntingtin increases toxicity

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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Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

21 January 2018

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Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

21 January 2018

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

21 January 2018

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Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release

21 January 2018

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Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

21 January 2018

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Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

21 January 2018

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Polyglutamine pathogenesis.

21 January 2018

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Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity

21 January 2018

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Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

21 January 2018

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A vector for expressing foreign genes in the brains and hearts of transgenic mice

21 January 2018

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Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

21 January 2018

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Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.

21 January 2018

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Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

21 January 2018

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

21 January 2018

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Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context

21 January 2018

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Widespread expression of Huntington's disease gene (IT15) protein product

21 January 2018

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Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies

21 January 2018

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Huntingtin contains a highly conserved nuclear export signal

21 January 2018

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Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

21 January 2018

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Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

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Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

21 January 2018

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Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins

21 January 2018

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Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes

21 January 2018

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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

21 January 2018

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Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

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Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein

21 January 2018

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Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7

21 January 2018

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

21 January 2018

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Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis

21 January 2018

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Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine

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Interaction of expanded polyglutamine stretches with nuclear transcription factors leads to aberrant transcriptional regulation in polyglutamine diseases

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Interaction of Huntington disease protein with transcriptional activator Sp1

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Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

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Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

21 January 2018

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Inhibition of tryptophan hydroxylase activity and decreased 5-HT1A receptor binding in a mouse model of Huntington's disease

21 January 2018

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The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor

21 January 2018

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The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis

21 January 2018

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Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

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CRE-mediated transcription is increased in Huntington's disease transgenic mice.

21 January 2018

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Huntington's disease: a synaptopathy?

21 January 2018

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A huntingtin-associated protein enriched in brain with implications for pathology

21 January 2018

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HIP-I: a huntingtin interacting protein isolated by the yeast two-hybrid system

21 January 2018

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HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain

21 January 2018

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The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis

21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDH175

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7 December 2017

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