(Q28594336)

31 January 2017

title

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

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31 January 2017

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GOA release 2020-03-11

P. F. Shelbourne

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31 January 2017

N. Killeen

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31 January 2017

R. F. Hevner

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31 January 2017

H. M. Johnston

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31 January 2017

L. Tecott

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31 January 2017

M. Lewandoski

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31 January 2017

M. Ennis

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31 January 2017

L. Ramirez

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31 January 2017

Z. Li

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31 January 2017

C. Iannicola

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31 January 2017

D. R. Littman

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31 January 2017

R. M. Myers

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31 January 2017

publication date

1 May 1999

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31 January 2017

published in

Human Molecular Genetics

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31 January 2017

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31 January 2017

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31 January 2017

page(s)

763–774

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

31 January 2017

cites work

1 reference

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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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The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease

21 January 2018

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Mitotic stability and meiotic variability of the (CAG)n repeat in the Huntington disease gene

21 January 2018

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Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat

21 January 2018

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Inactivation of the mouse Huntington's disease gene homolog Hdh

21 January 2018

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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

21 January 2018

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Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

21 January 2018

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

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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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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Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

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

21 January 2018

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Evidence from antibody studies that the CAG repeat in the Huntington disease gene is expressed in the protein

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

21 January 2018

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

21 January 2018

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Single sperm analysis of the trinucleotide repeats in the Huntington's disease gene: quantification of the mutation frequency spectrum

21 January 2018

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

21 January 2018

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Calbindin D28K as a marker for the degeneration of the striatonigral pathway in Huntington's disease

21 January 2018

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Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease

21 January 2018

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Changes in the visual system of monocularly sutured or enucleated cats demonstrable with cytochrome oxidase histochemistry

21 January 2018

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

21 January 2018

1 reference

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Behavioural abnormalities in male mice lacking neuronal nitric oxide synthase.

21 January 2018

1 reference

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Altered nociception, analgesia and aggression in mice lacking the receptor for substance P

21 January 2018

1 reference

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The clinical manifestation of mental disorder in Huntington's disease: a retrospective case record study of disease progression

21 January 2018

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Huntington's chorea. Investigation into the prevalence of this disease in the area covered by the North East Metropolitan Regional Hospital Board

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Impaired synaptic plasticity in mice carrying the Huntington's disease mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Increased trinucleotide repeat instability with advanced maternal age.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Transgenic mice harboring a full-length human mutant DRPLA gene exhibit age-dependent intergenerational and somatic instabilities of CAG repeats comparable with those in DRPLA patients.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Moderate intergenerational and somatic instability of a 55-CTG repeat in transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Stability of the Huntington disease (CAG)n repeat in a late onset form occuring on the Island of Crete

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Increased instability of intermediate alleles in families with sporadic Huntington disease compared to similar sized intermediate alleles in the general population

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Direct and crossover PCR amplification to facilitate Tn5supF-based sequencing of lambda phage clones

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Targeted disruption of the mouse apobec-1 gene abolishes apolipoprotein B mRNA editing and eliminates apolipoprotein B48

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Epithelial immaturity and multiorgan failure in mice lacking epidermal growth factor receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

Hypermutable myotonic dystrophy CTG repeats in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2F8.5.763

21 January 2018

Identifiers

DOI

10.1093/HMG/8.5.763

0 references

1 reference