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Huntington's disease: underlying molecular mechanisms and emerging concepts
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title
Huntington's disease: underlying molecular mechanisms and emerging concepts
(English)
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main subject
Huntington's disease
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author name string
John Labbadia
series ordinal
1
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stated in
Crossref
Richard I. Morimoto
series ordinal
2
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stated in
Crossref
language of work or name
English
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stated in
PubMed
publication date
August 2013
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stated in
PubMed
published in
Trends in Biochemical Sciences
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stated in
PubMed
volume
38
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stated in
PubMed
page(s)
378-85
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stated in
PubMed
issue
8
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PubMed
cites work
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Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression.
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Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease
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IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease
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Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function.
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Neuronal circuitry regulates the response of Caenorhabditis elegans to misfolded proteins
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28 September 2017
What have we learned from gene expression profiles in Huntington's disease?
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28 September 2017
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease
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Neuronal expression of TATA box-binding protein containing expanded polyglutamine in knock-in mice reduces chaperone protein response by impairing the function of nuclear factor-Y transcription factor
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Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration
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28 September 2017
Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative
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28 September 2017
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity
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28 September 2017
Energy deficit in Huntington disease: why it matters
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28 September 2017
Brain networks in Huntington disease
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28 September 2017
Neuronal degeneration in striatal transplants and Huntington's disease: potential mechanisms and clinical implications.
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28 September 2017
Proteasomal dysfunction in aging and Huntington disease
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28 September 2017
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28 September 2017
Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease
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28 September 2017
Identification of MOAG-4/SERF as a regulator of age-related proteotoxicity.
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28 September 2017
Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease
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28 September 2017
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
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28 September 2017
A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation
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PubMed Central
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28 September 2017
Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease
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28 September 2017
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease
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PubMed Central
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28 September 2017
Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice
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28 September 2017
Reduced IGF-1 signaling delays age-associated proteotoxicity in mice
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28 September 2017
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin
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PubMed Central
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28 September 2017
Multiple roles of HDAC inhibition in neurodegenerative conditions.
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28 September 2017
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin
1 reference
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PubMed Central
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28 September 2017
Brain-derived neurotrophic factor in neurodegenerative diseases
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28 September 2017
Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1
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PubMed Central
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28 September 2017
Polyglutamine neurodegeneration: protein misfolding revisited.
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28 September 2017
Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease.
1 reference
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PubMed Central
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28 September 2017
Regulation of the cellular heat shock response in Caenorhabditis elegans by thermosensory neurons
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28 September 2017
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking
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PubMed Central
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28 September 2017
Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor.
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
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28 September 2017
Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells
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28 September 2017
Progressive disruption of cellular protein folding in models of polyglutamine diseases.
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28 September 2017
Modulation of neurodegeneration by molecular chaperones
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PubMed Central
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28 September 2017
Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation
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PubMed Central
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28 September 2017
Unfolding the role of protein misfolding in neurodegenerative diseases
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PubMed Central
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28 September 2017
Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
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28 September 2017
Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets
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PubMed Central
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31 May 2018
Critical nucleus size for disease-related polyglutamine aggregation is repeat-length dependent
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
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31 May 2018
HTT haplotypes contribute to differences in Huntington disease prevalence between Europe and East Asia
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
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31 May 2018
The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation
1 reference
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PubMed Central
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31 May 2018
Acetylation targets mutant huntingtin to autophagosomes for degradation
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
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31 May 2018
In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets
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PubMed Central
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31 May 2018
Firefly luciferase mutants as sensors of proteome stress
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28 November 2018
Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism
1 reference
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PubMed Central
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28 November 2018
Global changes to the ubiquitin system in Huntington's disease.
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PubMed Central
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retrieved
28 November 2018
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
retrieved
28 November 2018
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
retrieved
28 November 2018
Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
retrieved
28 November 2018
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
retrieved
28 November 2018
Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3955166
retrieved
28 November 2018
Singles engage the RNA interference pathway
1 reference
stated in
PubMed
reference URL
https://pubmed.ncbi.nlm.nih.gov/23768628
retrieved
12 December 2020
based on heuristic
inferred from PubMed ID database lookup
Identifiers
DOI
10.1016/J.TIBS.2013.05.003
1 reference
stated in
Consolidated OpenCitations Corpus – April 2017
OpenCitations bibliographic resource ID
1018393
OpenCitations bibliographic resource ID
1018393
1 reference
stated in
Consolidated OpenCitations Corpus – April 2017
OpenCitations bibliographic resource ID
1018393
PMCID
3955166
1 reference
stated in
Consolidated OpenCitations Corpus – April 2017
OpenCitations bibliographic resource ID
1018393
PubMed ID
23768628
1 reference
stated in
Consolidated OpenCitations Corpus – April 2017
OpenCitations bibliographic resource ID
1018393
ResearchGate publication ID
239525039
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