(Q33650539)

28 July 2017

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title

Recent advances in understanding the pathogenesis of Huntington's disease (English)

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28 July 2017

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Reddy PH

1

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PubMed ID

10354600

28 July 2017

Williams M

series ordinal

2

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PubMed ID

10354600

28 July 2017

Tagle DA

series ordinal

3

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28 July 2017

1 June 1999

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28 July 2017

Trends in Neurosciences

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28 July 2017

22

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28 July 2017

6

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28 July 2017

248-255

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

28 July 2017

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Genetics and molecular biology of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

The expanding world of trinucleotide repeats

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

The expanding world of ataxins

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

The complex pathology of trinucleotide repeats

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

A polymorphic DNA marker genetically linked to Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Neuropathological classification of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Neuronal loss in the hippocampus in Huntington's disease: a comparison with HIV infection

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Huntington's disease in children. Neuropathologic study of four cases

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https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Morphometric studies of the neuropathological changes in choreatic diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Cellular localization of huntingtin in striatal and cortical neurons in rats: lack of correlation with neuronal vulnerability in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Partial characterisation of murine huntingtin and apparent variations in the subcellular localisation of huntingtin in human, mouse and rat brain.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Huntingtin interacts with a family of WW domain proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

Inactivation of the mouse Huntington's disease gene homolog Hdh

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

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%2FS0166-2236%2899%2901415-0

7 January 2021

Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

A cellular model that recapitulates major pathogenic steps of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2899%2901415-0

7 January 2021

10.1016/S0166-2236(99)01415-0

Identifiers

DOI

1 reference

28 July 2017

1 reference

PubMed ID

10354600