Dysfunction of the ubiquitin ligase Ube3a may be associated with synaptic pathophysiology in a mouse model of Huntington disease (Q36216036)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22787151%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

title

Dysfunction of the ubiquitin ligase Ube3a may be associated with synaptic pathophysiology in a mouse model of Huntington disease (English)

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7 February 2020

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5

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7 February 2020

author name string

Megha Maheshwari

1

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7 February 2020

Ananya Samanta

2

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7 February 2020

Swetha K Godavarthi

3

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7 February 2020

Rajarshi Mukherjee

4

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7 February 2020

language of work or name

English

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publication date

11 July 2012

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Journal of Biological Chemistry

7 February 2020

published in

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7 February 2020

volume

287

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7 February 2020

page(s)

29949-29957

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7 February 2020

issue

35

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Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin.

7 February 2020

cites work

1 reference

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Brief ampakine treatments slow the progression of Huntington's disease phenotypes in R6/2 mice

1 October 2017

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Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease

1 October 2017

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Cognitive and functional decline in Huntington's disease: dementia criteria revisited

1 October 2017

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Tissue-specific variation of Ube3a protein expression in rodents and in a mouse model of Angelman syndrome

1 October 2017

1 reference

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Genomic imprinting of experience-dependent cortical plasticity by the ubiquitin ligase gene Ube3a

1 October 2017

1 reference

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The Angelman Syndrome protein Ube3A regulates synapse development by ubiquitinating arc.

1 October 2017

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Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice

1 October 2017

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Co-chaperone CHIP stabilizes aggregate-prone malin, a ubiquitin ligase mutated in Lafora disease

1 October 2017

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Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease

1 October 2017

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Ube3a is required for experience-dependent maturation of the neocortex.

1 October 2017

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Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice

1 October 2017

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The ubiquitin ligase E6-AP is induced and recruited to aggresomes in response to proteasome inhibition and may be involved in the ubiquitination of Hsp70-bound misfolded proteins

1 October 2017

1 reference

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Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice

1 October 2017

1 reference

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Transcriptional signatures in Huntington's disease

1 October 2017

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Trinucleotide Repeat Disorders

1 October 2017

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Arc/Arg3.1 interacts with the endocytic machinery to regulate AMPA receptor trafficking

1 October 2017

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Arc/Arg3.1: linking gene expression to synaptic plasticity and memory

1 October 2017

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The roles of intracellular protein-degradation pathways in neurodegeneration

1 October 2017

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Diseases of unstable repeat expansion: mechanisms and common principles

1 October 2017

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Neurological aspects of the Angelman syndrome

1 October 2017

1 reference

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Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.

1 October 2017

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Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series

1 October 2017

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Recent advances in understanding the pathogenesis of polyglutamine diseases: involvement of molecular chaperones and ubiquitin-proteasome pathway.

1 October 2017

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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 October 2017

1 reference

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Transcriptional dysregulation in Huntington's disease

1 October 2017

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Genetics of Angelman syndrome

1 October 2017

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Impaired synaptic plasticity in mice carrying the Huntington's disease mutation

1 October 2017

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

1 October 2017

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UBE3A/E6-AP mutations cause Angelman syndrome

1 October 2017

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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 October 2017

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

1 October 2017

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Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease.

1 October 2017

1 reference

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Oxidative stress in Huntington's disease

29 June 2018

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Loss of dopaminergic neurons and resulting behavioural deficits in mouse model of Angelman syndrome

29 June 2018

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The ubiquitin ligase E6-AP promotes degradation of alpha-synuclein.

22 September 2018

1 reference

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E6-AP promotes misfolded polyglutamine proteins for proteasomal degradation and suppresses polyglutamine protein aggregation and toxicity

22 September 2018

1 reference

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Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

The Angelman syndrome ubiquitin ligase localizes to the synapse and nucleus, and maternal deficiency results in abnormal dendritic spine morphology

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Altered cortical glutamate receptor function in the R6/2 model of Huntington's disease

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease.

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Decreased expression of hypothalamic neuropeptides in Huntington disease transgenic mice with expanded polyglutamine-EGFP fluorescent aggregates.

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA and protein expression, plasma membrane association, interacting proteins, and phosphorylation

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation.

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation.

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3436189

Cognitive scores in carriers of Huntington's disease gene compared to noncarriers

22 September 2018

1 reference

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22 September 2018

Identifiers

DOI

10.1074/JBC.M112.371724

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22787151%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22787151%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

1 reference