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Lessons from animal models of Huntington's disease
scientific article
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instance of
scholarly article
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
review article
1 reference
stated in
Europe PubMed Central
title
Lessons from animal models of Huntington's disease
(English)
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
main subject
Huntington's disease
0 references
author name string
David C Rubinsztein
series ordinal
1
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
publication date
1 April 2002
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
published in
Trends in Genetics
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
volume
18
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
page(s)
202-209
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
issue
4
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
cites work
Huntington disease.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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The molecular biology of Huntington's disease
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Inactivation of the mouse Huntington's disease gene homolog Hdh
1 reference
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7 January 2021
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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
1 reference
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reference URL
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7 January 2021
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Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Age-dependent and tissue-specific CAG repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Neurological abnormalities in a knock-in mouse model of Huntington's disease
1 reference
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7 January 2021
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Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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inferred from DOI database lookup
The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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The actin-binding protein Hip1R associates with clathrin during early stages of endocytosis and promotes clathrin assembly in vitro
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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inferred from DOI database lookup
Wild-type huntingtin protects from apoptosis upstream of caspase-3.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
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
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death
1 reference
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Crossref
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
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Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
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Glutamine repeats and neurodegenerative diseases: molecular aspects
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
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Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
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.
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
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
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Mutant protein in Huntington disease is resistant to proteolysis in affected brain
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
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NMDA receptor losses in putamen from patients with Huntington's disease
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Mutant huntingtin enhances excitotoxic cell death.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Biochemical abnormalities and excitotoxicity in Huntington's disease brain.
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Mice transgenic for the Huntington's disease mutation are resistant to chronic 3-nitropropionic acid-induced striatal toxicity.
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
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
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Early alterations in gene expression and cell morphology in a mouse model of Huntington's disease.
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
CREB-binding protein sequestration by expanded polyglutamine
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity
1 reference
stated in
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Histone deacetylase inhibitors reduce polyglutamine toxicity
1 reference
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Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
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Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice
1 reference
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7 January 2021
based on heuristic
inferred from DOI database lookup
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
1 reference
stated in
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Genetic suppression of polyglutamine toxicity in Drosophila
1 reference
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https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Identification of genes that modify ataxin-1-induced neurodegeneration.
1 reference
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Expanded polyglutamine peptides alone are intrinsically cytotoxic and cause neurodegeneration in Drosophila
1 reference
stated in
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.
1 reference
stated in
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reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
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7 January 2021
based on heuristic
inferred from DOI database lookup
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Genotypes at the GluR6 kainate receptor locus are associated with variation in the age of onset of Huntington disease
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902625-7
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Identifiers
DOI
10.1016/S0168-9525(01)02625-7
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
PubMed ID
11932021
1 reference
stated in
Europe PubMed Central
PubMed ID
11932021
retrieved
3 August 2017
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