Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease. (Q43792416)

26 November 2017

Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease (English)

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12 May 2020

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12 May 2020

author name string

E Martín-Aparicio

1

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12 May 2020

A Yamamoto

2

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12 May 2020

R Hen

4

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J Avila

5

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J J Lucas

6

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language of work or name

English

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publication date

1 November 2001

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26 November 2017

Journal of Neuroscience

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volume

21

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page(s)

8772-8781

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issue

22

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Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

12 May 2020

cites work

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12 December 2020

Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production

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12 December 2020

Reversing neurodegeneration: a promise unfolds

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Homozygotes for Huntington's disease

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12 December 2020

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

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12 December 2020

Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

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12 December 2020

Decreased nuclear beta-catenin, tau hyperphosphorylation and neurodegeneration in GSK-3beta conditional transgenic mice

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12 December 2020

Decreased levels of proteasome activity and proteasome expression in aging spinal cord

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12 December 2020

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

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12 December 2020

Possible involvement of proteasome inhibition in aging: implications for oxidative stress.

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12 December 2020

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

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12 December 2020

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

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12 December 2020

Huntington's disease: the challenge for cell biologists

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12 December 2020

Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death

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12 December 2020

Mutational bias provides a model for the evolution of Huntington's disease and predicts a general increase in disease prevalence.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Impairment of the ubiquitin-proteasome system by protein aggregation

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12 December 2020

Primary culture of identified neurons from the visual cortex of postnatal rats.

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12 December 2020

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

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12 December 2020

Developmental regulation of type II calcium/calmodulin-dependent kinase isoforms in rat cerebellum

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12 December 2020

Phosphorylation of a neuronal-specific beta-tubulin isotype

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12 December 2020

Trinucleotide instability: a repeating theme in human inherited disorders

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12 December 2020

Tight control of gene expression in mammalian cells by tetracycline-responsive promoters

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12 December 2020

Development of inhibitory synapses among striatal neurons in vitro

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12 December 2020

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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12 December 2020

Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

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12 December 2020

Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

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12 December 2020

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

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12 December 2020

Neuropathological classification of Huntington's disease

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12 December 2020

SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein

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12 December 2020

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

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12 December 2020

Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract

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12 December 2020

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Genetic neurodegenerative diseases: the human illness and transgenic models

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12 December 2020

Calcium, free radicals, and excitotoxic neuronal death in primary cell culture

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12 December 2020

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

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12 December 2020

10.1523/JNEUROSCI.21-22-08772.2001

Identifiers

DOI

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6762274&resulttype=core&format=json

12 May 2020

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12 May 2020

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