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Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities.
scientific article published on 5 April 2018
Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities
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scholarly article
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stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
title
Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities
(English)
1 reference
stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
main subject
Huntington's disease
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author
Rachel Harding
series ordinal
1
1 reference
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Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
Yufeng Tong
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2
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stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
author name string
Yu-Feng Tong
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2
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Europe PubMed Central
PubMed ID
29620053
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4 May 2018
reference URL
http://europepmc.org/abstract/MED/29620053
language of work or name
English
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publication date
5 April 2018
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Europe PubMed Central
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5943907
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retrieved
22 March 2020
published in
Acta Pharmacologica Sinica
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Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
volume
39
1 reference
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Europe PubMed Central
PMCID
5943907
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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
issue
5
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Europe PubMed Central
PMCID
5943907
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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
page(s)
754-769
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Europe PubMed Central
PMCID
5943907
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retrieved
22 March 2020
exact match
https://scigraph.springernature.com/pub.10.1038/aps.2018.11
0 references
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AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington's disease.
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7 September 2018
Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A.
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7 September 2018
Identification of novel HSP90α/β isoform selective inhibitors using structure-based drug design. demonstration of potential utility in treating CNS disorders such as Huntington's disease
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7 September 2018
Huntington disease: natural history, biomarkers and prospects for therapeutics
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7 September 2018
Trehalose reverses cell malfunction in fibroblasts from normal and Huntington's disease patients caused by proteosome inhibition.
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7 September 2018
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response.
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7 September 2018
The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation
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7 September 2018
Sulforaphane enhances proteasomal and autophagic activities in mice and is a potential therapeutic reagent for Huntington's disease.
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7 September 2018
Rhes, a striatal-selective protein implicated in Huntington disease, binds beclin-1 and activates autophagy
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=5943907
retrieved
7 September 2018
Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies
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=5943907
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7 September 2018
A flexible polyglutamine hinge opens new doors for understanding huntingtin function
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=5943907
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7 September 2018
Expanded polyglutamine-containing N-terminal huntingtin fragments are entirely degraded by mammalian proteasomes
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=5943907
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7 September 2018
CAG size-specific risk estimates for intermediate allele repeat instability in Huntington disease
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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=5943907
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7 September 2018
miR-196a ameliorates phenotypes of Huntington disease in cell, transgenic mouse, and induced pluripotent stem cell models.
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=5943907
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7 September 2018
PGC-1α, mitochondrial dysfunction, and Huntington's disease
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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=5943907
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7 September 2018
Target validation using chemical probes
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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=5943907
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7 September 2018
MAP kinase phosphatase 1 (MKP-1/DUSP1) is neuroprotective in Huntington's disease via additive effects of JNK and p38 inhibition
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
A Strategy for Modulation of Enzymes in the Ubiquitin System
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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=5943907
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7 September 2018
Creation of an open-access, mutation-defined fibroblast resource for neurological disease research
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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=5943907
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7 September 2018
Dysfunction of the ubiquitin ligase Ube3a may be associated with synaptic pathophysiology in a mouse model of Huntington disease
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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=5943907
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7 September 2018
Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes
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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=5943907
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7 September 2018
Fibrillar α-synuclein and huntingtin exon 1 assemblies are toxic to the cells
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Dysregulation of synaptic proteins, dendritic spine abnormalities and pathological plasticity of synapses as experience-dependent mediators of cognitive and psychiatric symptoms in Huntington's disease.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Energy dysfunction in Huntington's disease: insights from PGC-1α, AMPK, and CKB.
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=5943907
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7 September 2018
The ubiquitin code
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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=5943907
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7 September 2018
Huntington disease and the huntingtin protein
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.
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=5943907
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7 September 2018
Bifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
A screen for enhancers of clearance identifies huntingtin as a heat shock protein 90 (Hsp90) client protein.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Inducing autophagy by rapamycin before, but not after, the formation of plaques and tangles ameliorates cognitive deficits
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
What have we learned from gene expression profiles in Huntington's disease?
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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=5943907
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7 September 2018
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Fighting neurodegeneration with rapamycin: mechanistic insights
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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=5943907
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7 September 2018
Acetylation regulates gluconeogenesis by promoting PEPCK1 degradation via recruiting the UBR5 ubiquitin ligase
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Kinase inhibitors modulate huntingtin cell localization and toxicity.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Development of target protein-selective degradation inducer for protein knockdown.
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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=5943907
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7 September 2018
EDD inhibits ATM-mediated phosphorylation of p53.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
AMPK and mTOR regulate autophagy through direct phosphorylation of Ulk1
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Regulation of the human papillomavirus type 18 E6/E6AP ubiquitin ligase complex by the HECT domain-containing protein EDD.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Proteasomal dysfunction in aging and Huntington disease
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Hsp70 and Hsp40 functionally interact with soluble mutant huntingtin oligomers in a classic ATP-dependent reaction cycle
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Enhancement of proteasome activity by a small-molecule inhibitor of USP14
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=5943907
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7 September 2018
Probing the probes: fitness factors for small molecule tools
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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=5943907
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7 September 2018
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Rapamycin protects against neuron death in in vitro and in vivo models of Parkinson's disease
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
TRIM9, a novel brain-specific E3 ubiquitin ligase, is repressed in the brain of Parkinson's disease and dementia with Lewy bodies
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Identification of the key structural motifs involved in HspB8/HspB6-Bag3 interaction
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Effects of partial suppression of parkin on huntingtin mutant R6/1 mice.
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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=5943907
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7 September 2018
Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism
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=5943907
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7 September 2018
Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Role of proteasomes in cellular regulation
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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=5943907
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7 September 2018
Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Polyubiquitin chains: functions, structures, and mechanisms
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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=5943907
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7 September 2018
Beneficial effects of rolipram in the R6/2 mouse model of Huntington's disease
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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=5943907
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7 September 2018
Novel targets for Huntington's disease in an mTOR-independent autophagy pathway
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
A novel brain-enriched E3 ubiquitin ligase RNF182 is up regulated in the brains of Alzheimer's patients and targets ATP6V0C for degradation
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
HspB8 and Bag3: a new chaperone complex targeting misfolded proteins to macroautophagy
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Celastrol inhibits polyglutamine aggregation and toxicity though induction of the heat shock response
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin.
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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=5943907
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7 September 2018
Profiling proteasome activity in tissue with fluorescent probes
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Roles of heat-shock protein 90 in maintaining and facilitating the neurodegenerative phenotype in tauopathies
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Proteasome activator enhances survival of Huntington's disease neuronal model cells
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein.
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Inositol and IP3 levels regulate autophagy: biology and therapeutic speculations.
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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=5943907
retrieved
7 September 2018
CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation
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=5943907
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7 September 2018
Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Bortezomib: proteasome inhibition as an effective anticancer therapy
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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=5943907
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7 September 2018
The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies
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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=5943907
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7 September 2018
A novel transgenic mouse model reveals deregulation of the ubiquitin-proteasome system in the heart by doxorubicin
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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=5943907
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7 September 2018
Small-molecule inhibition of proteasome and aggresome function induces synergistic antitumor activity in multiple myeloma
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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=5943907
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7 September 2018
Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.
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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=5943907
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7 September 2018
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes.
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=5943907
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7 September 2018
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 2018
Inefficient degradation of truncated polyglutamine proteins by the proteasome
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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=5943907
retrieved
7 September 2018
Proteasome degrades soluble expanded polyglutamine completely and efficiently
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=5943907
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7 September 2018
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
1 reference
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
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7 September 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
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PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5943907
retrieved
7 September 2018
Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
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=5943907
retrieved
7 September 2018
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
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=5943907
retrieved
7 September 2018
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
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=5943907
retrieved
7 September 2018
Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function.
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=5943907
retrieved
7 September 2018
The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans
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=5943907
retrieved
7 September 2018
Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy
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=5943907
retrieved
7 September 2018
Uncharged thioflavin-T derivatives bind to amyloid-beta protein with high affinity and readily enter the brain
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=5943907
retrieved
7 September 2018
Comparison of ARM and HEAT protein repeats
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=5943907
retrieved
7 September 2018
Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity
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=5943907
retrieved
7 September 2018
Protacs: chimeric molecules that target proteins to the Skp1-Cullin-F box complex for ubiquitination and degradation
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=5943907
retrieved
7 September 2018
Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease
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=5943907
retrieved
7 September 2018
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
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=5943907
retrieved
7 September 2018
Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular 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=5943907
retrieved
7 September 2018
Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
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=5943907
retrieved
7 September 2018
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
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=5943907
retrieved
7 September 2018
Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.
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=5943907
retrieved
7 September 2018
Huntington disease.
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=5943907
retrieved
7 September 2018
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
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=5943907
retrieved
7 September 2018
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
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=5943907
retrieved
7 September 2018
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
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=5943907
retrieved
7 September 2018
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic 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=5943907
retrieved
7 September 2018
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.
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=5943907
retrieved
7 September 2018
The TSH, T4, T3 and prolactin responses to consecutive infusions of a potent and stabilized thyrotrophin releasing hormone analogue, RX77368, in man.
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=5943907
retrieved
7 September 2018
Epidemiology of Huntington disease
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1038%2FAPS.2018.11
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Autophagy induction rescues toxicity mediated by proteasome inhibition
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1038%2FAPS.2018.11
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
mGluR5 antagonism increases autophagy and prevents disease progression in the zQ175 mouse model of Huntington's disease.
1 reference
stated in
Crossref
reference URL
https://api.crossref.org/works/10.1038%2FAPS.2018.11
retrieved
7 January 2021
based on heuristic
inferred from DOI database lookup
Identifiers
DOI
10.1038/APS.2018.11
1 reference
stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
PMCID
5943907
1 reference
stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
PubMed ID
29620053
1 reference
stated in
Europe PubMed Central
PMCID
5943907
reference URL
https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29620053%20AND%20SRC:MED&resulttype=core&format=json
retrieved
22 March 2020
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