Therapeutic Approaches for Inhibition of Protein Aggregation in Huntington's Disease

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Therapeutic Approaches for Inhibition of Protein Aggregation in Huntington's Disease is …
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scholarly articleQ13442814

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P356DOI10.5607/EN.2014.23.1.36
P8608Fatcat IDrelease_u7ogpne6z5a4bdel4bdxeda6d4
P932PMC publication ID3984955
P698PubMed publication ID24737938
P5875ResearchGate publication ID261745661

P2093author name stringKyong-Tai Kim
Sangjune Kim
P2860cites workThe Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcriptionQ22254119
A huntingtin-associated protein enriched in brain with implications for pathologyQ24303499
The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein AlfyQ24307759
Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicityQ24317318
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review seriesQ24537164
Misfolded proteins partition between two distinct quality control compartmentsQ24651093
Dual Action of ATP Hydrolysis Couples Lid Closure to Substrate Release into the Group II Chaperonin ChamberQ27666606
Structural basis for inhibition of the Hsp90 molecular chaperone by the antitumor antibiotics radicicol and geldanamycinQ27766755
Microtubule acetylation promotes kinesin-1 binding and transport.Q27919704
Repression of heat shock transcription factor HSF1 activation by HSP90 (HSP90 complex) that forms a stress-sensitive complex with HSF1Q28118304
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genesQ28189644
Loss of huntingtin-mediated BDNF gene transcription in Huntington's diseaseQ28202050
Normal huntingtin function: an alternative approach to Huntington's diseaseQ28281904
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotesQ28302701
Regulation of HSF1 function in the heat stress response: implications in aging and diseaseQ28307651
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubulesQ28590634
Trinucleotide Repeat DisordersQ29038716
Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state.Q52573641
Dynamic regulation of molecular chaperone gene expression in polyglutamine disease.Q52659334
Proteasome-dependent degradation of cytosolic chaperonin CCTQ73300148
Caspase-8 is required for cell death induced by expanded polyglutamine repeats.Q40961806
Genetic suppression of polyglutamine toxicity in DrosophilaQ41724127
The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregationQ42699018
Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanismQ43100835
Insoluble TATA-binding protein accumulation in Huntington's disease cortex.Q44280721
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.Q44286601
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approachQ44872348
Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's diseaseQ45298652
Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.Q45300254
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtinQ45301569
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.Q45302702
Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice.Q45303103
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's diseaseQ45304607
R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric miceQ45306743
Oligoproline effects on polyglutamine conformation and aggregationQ46830417
Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers.Q50715387
Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypesQ30536567
Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progressionQ31139684
Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in DrosophilaQ31142912
Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's diseaseQ33292417
Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative diseaseQ33526350
The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactionsQ33693921
Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregationQ33694463
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusionsQ33914031
Glutamine repeats and neurodegenerationQ33938576
Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.Q33948101
Genome-wide histone acetylation is altered in a transgenic mouse model of Huntington's diseaseQ34358457
Protein misfolding, amyloid formation, and neurodegeneration: a critical role for molecular chaperones?Q34747079
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's diseaseQ34763178
Chaperones and aging: role in neurodegeneration and in other civilizational diseasesQ34815474
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disordersQ34952853
Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrilsQ35169533
Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouseQ35345762
Protein folding revisited. A polypeptide chain at the folding ? misfolding ? nonfolding cross-roads: which way to go?Q35549177
Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperonesQ35674426
Modulation of neurodegeneration by molecular chaperonesQ35990274
A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouseQ36082278
TRiC's tricks inhibit huntingtin aggregationQ36998752
Prefoldin protects neuronal cells from polyglutamine toxicity by preventing aggregation formationQ37000637
Targeting H3K4 trimethylation in Huntington diseaseQ37088721
Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtinQ37157299
Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's diseaseQ37336253
Vaccinia-related kinase 2 mediates accumulation of polyglutamine aggregates via negative regulation of the chaperonin TRiC.Q37546880
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategiesQ37570639
Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington diseaseQ38345839
GRP78 counteracts cell death and protein aggregation caused by mutant huntingtin proteinsQ39366737
Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor.Q40010916
Histone deacetylase 6 inhibition compensates for the transport deficit in Huntington's disease by increasing tubulin acetylationQ40152899
Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse modelsQ40392026
Protein quality control: chaperones culling corrupt conformationsQ40400832
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c releaseQ40551652
Purification of polyglutamine aggregates and identification of elongation factor-1alpha and heat shock protein 84 as aggregate-interacting proteins.Q40692086
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.Q40704833
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cellsQ40886004
P275copyright licenseCreative Commons Attribution-NonCommercial 3.0 UnportedQ18810331
P6216copyright statuscopyrightedQ50423863
P433issue1
P407language of work or nameEnglishQ1860
P921main subjectprotein aggregationQ7251455
Huntington's diseaseQ190564
P304page(s)36-44
P577publication date2014-03-01
P1433published inExperimental neurobiologyQ26842060
P1476titleTherapeutic Approaches for Inhibition of Protein Aggregation in Huntington's Disease
P478volume23

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cites work (P2860)
Q58608836Aggregated SOD1 causes selective death of cultured human motor neurons
Q36913723Alternative mitochondrial electron transfer for the treatment of neurodegenerative diseases and cancers: Methylene blue connects the dots.
Q91670445Brain Microbiota in Huntington's Disease Patients
Q38421354Car drivers with dementia: Different complications due to different etiologies?
Q52350721Fractionation for Resolution of Soluble and Insoluble Huntingtin Species.
Q45299657Hope for Huntington's disease? A novel approach for disease modification
Q47133162Mammalian display screening of diverse cystine-dense peptides for difficult to drug targets.
Q64912813Novel targets for Huntington's disease: future prospects.
Q26828757On the role of 4-hydroxynonenal in health and disease
Q56770267The role of Twist1 in mutant huntingtin-induced transcriptional alterations and neurotoxicity

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