Huntington's disease: translating a CAG repeat into a pathogenic mechanism (Q41235542)

28 September 2017

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title

Huntington's disease: translating a CAG repeat into a pathogenic mechanism (English)

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28 September 2017

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2

J F Gusella

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28 September 2017

M E MacDonald

1

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28 September 2017

1 October 1996

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Current Opinion in Neurobiology

28 September 2017

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28 September 2017

6

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28 September 2017

5

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28 September 2017

638-643

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Huntington's disease. Pathogenesis and management

28 September 2017

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Neurobiology of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Neuropathological classification of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Aging, energy, and oxidative stress in neurodegenerative diseases.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

1 reference

7 January 2021

Molecular genetics of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Huntington's disease: CAG genetics expands neurobiology

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Huntington's disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

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%2FS0959-4388%2896%2980097-3

7 January 2021

Normal and expanded Huntington's disease gene alleles produce distinguishable proteins due to translation across the CAG repeat.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Abnormal gene product identified in Huntington's disease lymphocytes and brain

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Mouse Huntington's disease gene homolog (Hdh).

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

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%2FS0959-4388%2896%2980097-3

7 January 2021

Expression of the Huntington disease gene in rodents: cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Comparative sequence analysis of the human and pufferfish Huntington's disease genes

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

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%2FS0959-4388%2896%2980097-3

7 January 2021

Inactivation of the mouse Huntington's disease gene homolog Hdh

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

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%2FS0959-4388%2896%2980097-3

7 January 2021

Homozygotes for Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Human genetic diseases due to codon reiteration: relationship to an evolutionary mechanism

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Huntingtin-associated protein (HAP1): discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Alternative excitotoxic hypotheses

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Unique features of the CAG repeats in Machado-Joseph disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

Does homozygosity advance the onset of dentatorubral-pallidoluysian atrophy?

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980097-3

7 January 2021

10.1016/S0959-4388(96)80097-3

Identifiers

DOI

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28 September 2017

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PubMed ID

8937828