Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients. (Q36916483)

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scientific article published on 17 July 2008

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English	Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients.	scientific article published on 17 July 2008

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scholarly article

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11 January 2020

Compensatory changes in the ubiquitin-proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients (English)

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11 January 2020

Huntington's disease

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ubiquitin-proteasome system

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3

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11 January 2020

author name string

Hyemyung Seo

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11 January 2020

Woori Kim

2

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17 July 2008

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11 January 2020

Human Molecular Genetics

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11 January 2020

17

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11 January 2020

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11 January 2020

3144-3153

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11 January 2020

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

1 reference

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Neuroinflammation mediated by IL-1beta increases susceptibility of dopamine neurons to degeneration in an animal model of Parkinson's disease

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29 September 2017

Huntington's disease: pathological mechanisms and therapeutic strategies

1 reference

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29 September 2017

Selective degeneration in YAC mouse models of Huntington disease

1 reference

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Proteasome activator enhances survival of Huntington's disease neuronal model cells

1 reference

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Integrative hypothesis for Huntington's disease: a brief review of experimental evidence.

1 reference

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29 September 2017

Mutant huntingtin impairs the post-Golgi trafficking of brain-derived neurotrophic factor but not its Val66Met polymorphism.

1 reference

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29 September 2017

Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease

1 reference

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Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin

1 reference

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3-Nitropropionic acid: a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease.

1 reference

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The ubiquitin-proteasome system and neurodegenerative disorders

1 reference

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Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease.

1 reference

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Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease.

1 reference

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29 September 2017

Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system

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29 September 2017

Environmental enrichment rescues protein deficits in a mouse model of Huntington's disease, indicating a possible disease mechanism.

1 reference

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Alzheimer's disease and Down's syndrome: roles of APP, trophic factors and ACh.

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29 September 2017

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

1 reference

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29 September 2017

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

1 reference

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29 September 2017

Expression of brain-derived neurotrophic factor in cortical neurons is regulated by striatal target area.

1 reference

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29 September 2017

Down-regulation of the neurotrophin receptor TrkB following ligand binding. Evidence for an involvement of the proteasome and differential regulation of TrkA and TrkB.

1 reference

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29 September 2017

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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29 September 2017

Distribution and retrograde transport of trophic factors in the central nervous system: functional implications for the treatment of neurodegenerative diseases

1 reference

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29 September 2017

Neurochemistry and toxin models in Huntington's disease.

1 reference

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Cortical neurons express nerve growth factor receptors in advanced age and Alzheimer disease

1 reference

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Chapter 26 Preparation and Characterization of Mitochondria and Submitochondrial Particles of Rat Liver and Liver-Derived Tissues

1 reference

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29 September 2017

Deficits of glutamate transmission in the striatum of toxic and genetic models of Huntington's disease

1 reference

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27 June 2018

Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.

1 reference

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27 June 2018

Transgenic models of Huntington's disease

1 reference

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27 June 2018

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2556853

27 June 2018

Expanded-polyglutamine huntingtin protein suppresses the secretion and production of a chemokine (CCL5/RANTES) by astrocytes

1 reference

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4 September 2018

Microglial activation in presymptomatic Huntington's disease gene carriers

1 reference

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Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice

1 reference

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4 September 2018

Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors.

1 reference

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4 September 2018

Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.

1 reference

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4 September 2018

Oral uridine pro-drug PN401 is neuroprotective in the R6/2 and N171-82Q mouse models of Huntington's disease

1 reference

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Combined minocycline plus pyruvate treatment enhances effects of each agent to inhibit inflammation, oxidative damage, and neuronal loss in an excitotoxic animal model of Huntington's disease

1 reference

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1 reference

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Dopamine release is severely compromised in the R6/2 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=2556853

4 September 2018

Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain

1 reference

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4 September 2018

Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice

1 reference

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4 September 2018

Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 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=2556853

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Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin

1 reference

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Progressive loss of BDNF in a mouse model of Huntington's disease and rescue by BDNF delivery

1 reference

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1 reference

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Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

1 reference

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Generalized brain and skin proteasome inhibition in Huntington's disease

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

1 reference

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1 reference

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Neuronal vulnerability following inhibition of mitochondrial complex II: a possible ionic mechanism for Huntington's disease

1 reference

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NMDA receptor function in mouse models of Huntington disease

1 reference

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4 September 2018

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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4 September 2018

Mitochondrial impairment reduces the threshold for in vivo NMDA-mediated neuronal death in the striatum

1 reference

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4 September 2018

Age dependence of striatal neuronal death caused by mitochondrial dysfunction

1 reference

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Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid.

1 reference

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Identifiers

10.1093/HMG/DDN211

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11 January 2020

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11 January 2020

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11 January 2020

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