(Q41682897)

7 October 2017

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title

Transgenic mouse models of neurodegenerative disease caused by CAG/polyglutamine expansions (English)

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7 October 2017

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7 October 2017

Davies SW

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7 October 2017

publication date

1 November 1997

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Trends in Molecular Medicine

7 October 2017

published in

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7 October 2017

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7 October 2017

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7 October 2017

page(s)

508-515

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Transgenic models of Huntington's disease

7 October 2017

cites work

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7 January 2021

When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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7 January 2021

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

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7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Abnormal gene product identified in hereditary dentatorubral–pallidoluysian atrophy (DRPLA) brain

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Inactivation of the mouse Huntington's disease gene homolog Hdh

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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7 January 2021

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

1 reference

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7 January 2021

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

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7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

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7 January 2021

Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Trinucleotide repeat length instability and age of onset in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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7 January 2021

Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I.

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7 January 2021

Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm.

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Somatic mosaicism of CAG repeat in dentatorubral-pallidoluysian atrophy (DRPLA)

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Gametic and somatic tissue-specific heterogeneity of the expanded SCA1 CAG repeat in spinocerebellar ataxia type 1.

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Differential pattern in tissue-specific somatic mosaicism of expanded CAG trinucleotide repeats in dentatorubral-pallidoluysian atrophy, Machado-Joseph disease, and X-linked recessive spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype

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7 January 2021

Mouse Huntington's disease gene homolog (Hdh).

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Sequence of the murine Huntington disease gene: evidence for conservation, alternate splicing and polymorphism in a triplet (CCG) repeat [corrected]

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Neuropathological classification of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Hypermutable myotonic dystrophy CTG repeats in transgenic mice

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

Myotonic dystrophy reviewed: back to the future?

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https://api.crossref.org/works/10.1016%2FS1357-4310%2897%2901142-8

7 January 2021

10.1016/S1357-4310(97)01142-8

Identifiers

DOI

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7 October 2017

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