(Q34001953)

30 July 2017

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title

Transcriptional dysregulation in Huntington's disease (English)

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transcriptional dysregulation

30 July 2017

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1 reference

Cha JH

1

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30 July 2017

1 September 2000

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30 July 2017

Trends in Neurosciences

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30 July 2017

23

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30 July 2017

387-392

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30 July 2017

9

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https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

30 July 2017

1 reference

7 January 2021

The functional anatomy of basal ganglia disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2FS0166-2236%2800%2901609-X

7 January 2021

Characterization and localization of the Huntington disease gene product

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Expression of normal and mutant huntingtin in the developing brain

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Trinucleotide repeats in neurogenetic disorders

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The complex pathology of trinucleotide repeats

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Trinucleotide repeats in neurologic diseases: an hypothesis concerning the pathogenesis of Huntington's disease, Kennedy's disease, and spinocerebellar ataxia type I.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Ataxin-3 is transported into the nucleus and associates with the nuclear matrix

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Heterogeneous intracellular localization and expression of ataxin-3.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Evidence for a repressive function of the long polyglutamine tract in the human androgen receptor: possible pathogenetic relevance for the (CAG)n-expanded neuronopathies

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Transcriptional activation by the androgen receptor in X-linked spinal and bulbar muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Transcriptional activation modulated by homopolymeric glutamine and proline stretches

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Trinucleotide repeats and long homopeptides in genes and proteins associated with nervous system disease and development

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Evidence for a recruitment and sequestration mechanism in Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

A neurological disease caused by an expanded CAG trinucleotide repeat in the TATA-binding protein gene: a new polyglutamine disease?

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

The influence of huntingtin protein size on nuclear localization and cellular toxicity.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

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%2800%2901609-X

7 January 2021

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

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%2800%2901609-X

7 January 2021

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

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%2800%2901609-X

7 January 2021

Nuclear targeting of mutant Huntingtin increases toxicity

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https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Huntingtin interacts with a family of WW domain proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Huntingtin: a single bait hooks many species

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Huntingtin interacts with cystathionine beta-synthase

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Are there multiple pathways in the pathogenesis of Huntington's disease?

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Neurodegenerative diseases and transglutaminase

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

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%2800%2901609-X

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%2800%2901609-X

7 January 2021

Transgenic models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The ubiquitin-proteasome pathway is required for processing the NF-kappa B1 precursor protein and the activation of NF-kappa B

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

A proteasome inhibitor prevents activation of NF-kappa B and stabilizes a newly phosphorylated form of I kappa B-alpha that is still bound to NF-kappa B

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

p53-dependent cell cycle arrest induced by N-acetyl-L-leucinyl-L-leucinyl-L-norleucinal in platelet-derived growth factor-stimulated human fibroblasts

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Stabilization of p53 by adenovirus E1A occurs through its amino-terminal region by modification of the ubiquitin-proteasome pathway

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Functions of the MDM2 oncoprotein

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The ubiquitin-proteasome proteolytic pathway

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Ubiquitin, proteasomes, and the regulation of intracellular protein degradation

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Control of gene expression by proteolysis

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Nuclear factor-kappa B contributes to excitotoxin-induced apoptosis in rat striatum.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Kainic acid-induced apoptosis in rat striatum is associated with nuclear factor-kappaB activation

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The tyrosine kinase negative regulator c-Cbl as a RING-type, E2-dependent ubiquitin-protein ligase

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Polyglutamine pathogenesis.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

The localization and interactions of huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease: a detailed cellular in situ hybridization study

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

A complex program of striatal gene expression induced by dopaminergic stimulation.

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

D1 and D2 dopamine receptor-regulated gene expression of striatonigral and striatopallidal neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Recent advances in understanding the pathogenesis of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

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%2800%2901609-X

7 January 2021

Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

Distinct molecular mechanisms and divergent endocytotic pathways of AMPA receptor internalization

1 reference

https://api.crossref.org/works/10.1016%2FS0166-2236%2800%2901609-X

7 January 2021

10.1016/S0166-2236(00)01609-X

Identifiers

DOI

1 reference

30 July 2017

1 reference

PubMed ID

10941183