(Q28593520)

1

1 reference

31 January 2017

Jon D. Wood

4

1 reference

31 January 2017

William J. Herring

5

1 reference

31 January 2017

Valina L. Dawson

Journal of Biological Chemistry

8

1 reference

31 January 2017

language of work or name

English

0 references

publication date

11 April 2003

1 reference

31 January 2017

1 reference

31 January 2017

278

1 reference

31 January 2017

15

1 reference

31 January 2017

13047–13055

1 reference

31 January 2017

Unusual form of cerebellar ataxia; combined dentato-rubral and pallido-Luysian degeneration

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA).

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

DNA analysis in hereditary dentatorubral-pallidoluysian atrophy: correlation between CAG repeat length and phenotypic variation and the molecular basis of anticipation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Novel triplet repeat containing genes in human brain: cloning, expression, and length polymorphisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Expansion explosion: new clues to the pathogenesis of repeat expansion neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

DRPLA gene (atrophin-1) sequence and mRNA expression in human brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Expression of dentatorubral-pallidoluysian atrophy (DRPLA) proteins in patients.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Expression and distribution of the dentatorubral-pallidoluysian atrophy gene product (atrophin-1/drplap) in neuronal and non-neuronal tissues.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Adenovirus-mediated expression of mutant DRPLA proteins with expanded polyglutamine stretches in neuronally differentiated PC12 cells. Preferential intranuclear aggregate formation and apoptosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Nuclear targeting of mutant Huntingtin increases toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Site-directed mutagenesis by overlap extension using the polymerase chain reaction

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Dentatorubral-pallidoluysian atrophy protein interacts through a proline-rich region near polyglutamine with the SH3 domain of an insulin receptor tyrosine kinase substrate

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Mutant protein in Huntington disease is resistant to proteolysis in affected brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Polyglutamine repeat length-dependent proteolysis of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Atrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Interaction between neuronal intranuclear inclusions and promyelocytic leukemia protein nuclear and coiled bodies in CAG repeat diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Reduction of Purkinje cell pathology in SCA1 transgenic mice by p53 deletion.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M211224200