(Q48357496)

10 February 2018

review article

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title

Transgenic mice in the study of polyglutamine repeat expansion diseases. (English)

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10 February 2018

author name string

Bates GP

series ordinal

1

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10 February 2018

Mangiarini L

series ordinal

2

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10 February 2018

Davies SW

series ordinal

3

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10 February 2018

publication date

1 October 1998

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10 February 2018

published in

Brain Pathology

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10 February 2018

volume

8

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10 February 2018

issue

4

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10 February 2018

page(s)

699-714

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10 February 2018

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

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Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

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Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion

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Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

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Inactivation of the mouse Huntington's disease gene homolog Hdh

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

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Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

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Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

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Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

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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CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

21 January 2018

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Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

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Huntingtin immunoreactivity in the rat neostriatum: differential accumulation in projection and interneurons.

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Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats

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

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

21 January 2018

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The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)

21 January 2018

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Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p

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

21 January 2018

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Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.

21 January 2018

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Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

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Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

21 January 2018

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Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.

21 January 2018

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Nuclear membrane indentations in Huntington's chorea

21 January 2018

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When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

21 January 2018

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT.

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Huntingtin localization in brains of normal and Huntington's disease patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.1998.TB00196.X

10.1111/J.1750-3639.1998.TB00196.X

21 January 2018

Identifiers

DOI

1 reference

10 February 2018

1 reference

10 February 2018