Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. (Q40661429)

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1 November 2019

title

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12657678%20AND%20SRC:MED&resulttype=core&format=json

1 November 2019

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Zhao-Xue Yu

1

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1 November 2019

Shi-Hua Li

2

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1 November 2019

Joy Evans

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1 November 2019

Ajay Pillarisetti

4

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1 November 2019

He Li

5

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1 November 2019

Xiao-Jiang Li

6

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1 November 2019

language of work or name

English

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publication date

1 March 2003

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1 November 2019

published in

Journal of Neuroscience

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1 November 2019

volume

23

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1 November 2019

issue

6

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1 November 2019

page(s)

2193-2202

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

1 November 2019

cites work

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

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12 December 2020

Energetics in the pathogenesis of neurodegenerative diseases

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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

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

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12 December 2020

Early alterations in gene expression and cell morphology in a mouse model of Huntington's disease.

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12 December 2020

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

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12 December 2020

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

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12 December 2020

Neurological abnormalities in a knock-in mouse model of Huntington's disease

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12 December 2020

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

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12 December 2020

Beyond the Qs in the polyglutamine diseases

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12 December 2020

Impairment of the ubiquitin-proteasome system by protein aggregation

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12 December 2020

Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

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12 December 2020

A simple stereologic method for analysis of cerebral cortical microvessels using image analysis

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12 December 2020

Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation

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12 December 2020

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.

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12 December 2020

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease

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12 December 2020

Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

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12 December 2020

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

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12 December 2020

Polyglutamine aggregates stimulate ER stress signals and caspase-12 activation

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12 December 2020

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

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12 December 2020

The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

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12 December 2020

Calpain activation in Huntington's disease.

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12 December 2020

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

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12 December 2020

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

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12 December 2020

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

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12 December 2020

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Degeneration of pyramidal projection neurons in Huntington's disease cortex

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12 December 2020

Atrophy of the hypothalamic lateral tuberal nucleus in Huntington's disease

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12 December 2020

Neuropathological classification of Huntington's disease

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12 December 2020

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

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12 December 2020

In situ evidence for DNA fragmentation in Huntingtonʼs disease striatum and Alzheimerʼs disease temporal lobes

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12 December 2020

DNA End Labeling (TUNEL) in Huntington's Disease and Other Neuropathological Conditions

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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

The cortical neuritic pathology of Huntington's disease

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12 December 2020

Early loss of neostriatal striosome neurons in Huntington's disease

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12 December 2020

Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington's disease: a quantitative Golgi study.

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12 December 2020

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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12 December 2020

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

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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

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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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

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

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12 December 2020

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

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12 December 2020

10.1523/JNEUROSCI.23-06-02193.2003

Identifiers

DOI

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1 November 2019

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1 November 2019

PubMed publication ID

12657678

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