Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease (Q45299022)

11 December 2017

title

Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease (English)

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11 December 2017

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5

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4

Blair R Leavitt

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11 December 2017

3

Elizabeth J Slow

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11 December 2017

Jeremy M Van Raamsdonk

series ordinal

1

object named as

Jeremy M Van Raamsdonk

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11 December 2017

Zoe Murphy

2

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11 December 2017

publication date

8 November 2005

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11 December 2017

published in

Human Molecular Genetics

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11 December 2017

volume

14

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11 December 2017

issue

24

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11 December 2017

page(s)

3823-3835

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Glutamine repeats and neurodegeneration

11 December 2017

cites work

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Preferential loss of striato-external pallidal projection neurons in presymptomatic Huntington's disease

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Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

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Huntington's disease gene (IT15) is widely expressed in human and rat tissues

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Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

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Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease.

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Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

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

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

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Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

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Nuclear targeting of mutant Huntingtin increases toxicity

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

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

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Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

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In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease

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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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Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

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

21 January 2018

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21 January 2018

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Analysis of the subcellular localization of huntingtin with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin: comparison with the distribution of huntingtin in Huntington's disease autopsy brain

21 January 2018

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Huntingtin contains a highly conserved nuclear export signal

21 January 2018

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Polyglutamine expansion of huntingtin impairs its nuclear export

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

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21 January 2018

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Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy

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

21 January 2018

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Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

21 January 2018

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Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

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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Neuronal intranuclear inclusions and neuropil aggregates in HdhCAG(150) knockin mice

21 January 2018

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

21 January 2018

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Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

21 January 2018

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Cortical and striatal neurone number in Huntington's disease

21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDI407

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11 December 2017

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