(Q28728849)

English

Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation

scientific article (publication date: 2012)

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scholarly article

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Temporal separation of aggregation and ubiquitination during early inclusion formation in transgenic mice carrying the Huntington's disease mutation (English)

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Huntington's disease

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protein ubiquitination

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based on heuristic

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3

object named as

A Jennifer Morton

author given names

A Jennifer

author last names

Morton

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PubMed publication ID

8 April 2024

author name string

Belvin Gong

1

author given names

Belvin

author last names

Gong

1 reference

PubMed publication ID

8 April 2024

Catherine Kielar

2

author given names

Catherine

author last names

Kielar

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PubMed publication ID

8 April 2024

language of work or name

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publication date

2012

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7

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7

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e41450

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copyright license

Creative Commons Attribution 4.0 International

24 July 2012

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April 2022 Public Data File from Crossref

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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

Expression of normal and mutant huntingtin in the developing brain

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

17 March 2017

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

7 April 2017

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://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

7 April 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

7 April 2017

Differential electrophysiological changes in striatal output neurons in Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

A compact beta model of huntingtin toxicity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Antibody therapy in neurodegenerative disease

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Alterations in cortical excitation and inhibition in genetic mouse models of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Neuroanatomic profile of polyglutamine immunoreactivity in Huntington disease brains.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

The R6 lines of transgenic mice: a model for screening new therapies for Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Mutant huntingtin can paradoxically protect neurons from death

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

CAG repeat disorder models and human neuropathology: similarities and differences

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Neuronal intranuclear and neuropil inclusions for pathological assessment of Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

A similar impairment in CA3 mossy fibre LTP in the R6/2 mouse model of Huntington's disease and in the complexin II knockout mouse

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

CREB-binding protein sequestration by expanded polyglutamine

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Intraneuronal aggregate formation and cell death after viral expression of expanded polyglutamine tracts in the adult rat brain.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

DNA analysis of Huntington's disease: five years of experience in Germany, Austria, and Switzerland

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Amyloid diseases: abnormal protein aggregation in neurodegeneration

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Homozygotes for Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

Homozygote for Huntington disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

3 June 2018

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

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

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/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Progressive formation of inclusions in the striatum and hippocampus of mice transgenic for the human Huntington's disease mutation

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Analysis of protein aggregation kinetics.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Nucleation of huntingtin aggregation in cells

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Stochastic kinetics of intracellular huntingtin aggregate formation.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6/2 mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Global changes to the ubiquitin system in Huntington's disease.

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0041450

21 January 2018

Aggregation in Huntington's disease: insights through modelling

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Huntington aggregates may not predict neuronal death in Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Homozygosity in Huntington's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3404089

27 November 2018

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/22848498

12 December 2020

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inferred from PubMed ID database lookup

Identifiers

10.1371/JOURNAL.PONE.0041450

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Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

2012PLoSO...741450G

0 references

OpenCitations bibliographic resource ID

1 reference

Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

PMC publication ID

1 reference

Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

PubMed publication ID

1 reference

Consolidated OpenCitations Corpus – April 2017

OpenCitations bibliographic resource ID

ResearchGate publication ID

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