(Q35764321)

10 August 2017

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title

Role of heat shock proteins during polyglutamine neurodegeneration: mechanisms and hypothesis (English)

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10 August 2017

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Andreas Wyttenbach

1

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10 August 2017

publication date

1 January 2004

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Journal of Molecular Neuroscience

10 August 2017

published in

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10 August 2017

volume

23

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10 August 2017

issue

1-2

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10 August 2017

page(s)

69-96

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Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor

10 August 2017

cites work

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12 December 2020

Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length

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12 December 2020

Modulation of Akt kinase activity by binding to Hsp90

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12 December 2020

Role of the heat shock response and molecular chaperones in oncogenesis and cell death

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12 December 2020

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

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12 December 2020

Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

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12 December 2020

Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

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12 December 2020

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

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12 December 2020

Evidence for a recruitment and sequestration mechanism in Huntington's disease

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12 December 2020

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

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12 December 2020

Polyglutamine expansion induces a protein-damaging stress connecting heat shock protein 70 to the JNK pathway

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12 December 2020

Function and regulation of CREB family transcription factors in the nervous system

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12 December 2020

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

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12 December 2020

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

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12 December 2020

Dynamic remodeling of transcription complexes by molecular chaperones

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12 December 2020

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

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12 December 2020

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

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12 December 2020

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

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12 December 2020

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

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12 December 2020

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

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12 December 2020

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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12 December 2020

Genetic suppression of polyglutamine toxicity in Drosophila

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12 December 2020

Identification of MAPKAP kinase 2 as a major enzyme responsible for the phosphorylation of the small mammalian heat shock proteins

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12 December 2020

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12 December 2020

Regulation of Hsp27 oligomerization, chaperone function, and protective activity against oxidative stress/tumor necrosis factor alpha by phosphorylation

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12 December 2020

Mitochondrial reactive oxygen species in cell death signaling

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12 December 2020

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

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12 December 2020

Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.

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12 December 2020

Role of the chaperone protein Hsp104 in propagation of the yeast prion-like factor [psi+]

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12 December 2020

Review: mechanisms of disaggregation and refolding of stable protein aggregates by molecular chaperones

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12 December 2020

Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

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12 December 2020

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

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12 December 2020

Heat-shock protein 70 inhibits apoptosis by preventing recruitment of procaspase-9 to the Apaf-1 apoptosome

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12 December 2020

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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12 December 2020

Structure and functions of the 20S and 26S proteasomes

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12 December 2020

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

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12 December 2020

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

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12 December 2020

Ubiquitin-dependent degradation of certain protein substrates in vitro requires the molecular chaperone Hsc70

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12 December 2020

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

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12 December 2020

Aggresomes: a cellular response to misfolded proteins

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12 December 2020

Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

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12 December 2020

Mouse heat shock transcription factor 1 deficiency alters cardiac redox homeostasis and increases mitochondrial oxidative damage

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12 December 2020

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

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12 December 2020

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

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12 December 2020

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

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12 December 2020

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

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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

Raised intracellular glucose concentrations reduce aggregation and cell death caused by mutant huntingtin exon 1 by decreasing mTOR phosphorylation and inducing autophagy

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12 December 2020

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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12 December 2020

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12 December 2020

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

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12 December 2020

Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components

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12 December 2020

Small stress proteins: chaperones that act as regulators of intracellular redox state and programmed cell death

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12 December 2020

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

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12 December 2020

Hsp105alpha suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity

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12 December 2020

Gene Expression Profiling in Ataxin-3 Expressing Cell Lines Reveals Distinct Effects of Normal and Mutant Ataxin-3

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12 December 2020

Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

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12 December 2020

Inhibitors of the proteasome block the degradation of most cell proteins and the generation of peptides presented on MHC class I molecules

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12 December 2020

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

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12 December 2020

Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis

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12 December 2020

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

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12 December 2020

Nitric oxide and nitric oxide synthase in Huntington's disease

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12 December 2020

Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression

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12 December 2020

Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

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12 December 2020

The role of chaperones in polyglutamine disease

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12 December 2020

Neurotrophin signal transduction in the nervous system

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12 December 2020

Extended polyglutamine selectively interacts with caspase-8 and -10 in nuclear aggregates

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12 December 2020

Polyglutamine protein aggregates are dynamic

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12 December 2020

The sizes of peptides generated from protein by mammalian 26 and 20 S proteasomes. Implications for understanding the degradative mechanism and antigen presentation

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12 December 2020

CREB-binding protein sequestration by expanded polyglutamine

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12 December 2020

Oxidative stress in Huntington's disease

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12 December 2020

Polyglutamine protein aggregation and toxicity are linked to the cellular stress response

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12 December 2020

Breaking the mitochondrial barrier

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12 December 2020

Aggresomes, inclusion bodies and protein aggregation

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12 December 2020

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

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12 December 2020

Hsp72 functions as a natural inhibitory protein of c-Jun N-terminal kinase

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12 December 2020

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice

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12 December 2020

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

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12 December 2020

Polyglutamine disease and neuronal cell death.

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12 December 2020

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

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12 December 2020

Protein aggregation in Huntington's and Parkinson's disease: implications for therapy.

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12 December 2020

Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils

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12 December 2020

Energetics in the pathogenesis of neurodegenerative diseases

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12 December 2020

Orchestrated response: a symphony of transcription factors for gene control

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12 December 2020

DNA damage induced by polyglutamine-expanded proteins

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12 December 2020

Heat shock protein hsp72 is a negative regulator of apoptosis signal-regulating kinase 1.

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12 December 2020

Glutamine repeats and neurodegenerative diseases: molecular aspects

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12 December 2020

Negative regulation of cytochrome c-mediated oligomerization of Apaf-1 and activation of procaspase-9 by heat shock protein 90

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12 December 2020

Alterations in the mouse and human proteome caused by Huntington's disease

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12 December 2020

When more is less: pathogenesis of glutamine repeat neurodegenerative diseases

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12 December 2020

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

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12 December 2020

Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin

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12 December 2020

Poly-ubiquitin Binding by the Polyglutamine Disease Protein Ataxin-3 Links Its Normal Function to Protein Surveillance Pathways

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12 December 2020

Ubiquitin-dependent protein degradation

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12 December 2020

Molecular chaperones in cellular protein folding

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12 December 2020

Inhibition of Daxx-mediated apoptosis by heat shock protein 27

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12 December 2020

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

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12 December 2020

Activation of MLK2-mediated signaling cascades by polyglutamine-expanded huntingtin

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12 December 2020

Stress-mediated signaling in PC12 cells - the role of the small heat shock protein, Hsp27, and Akt in protecting cells from heat stress and nerve growth factor withdrawal

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12 December 2020

Protein turnover: a CHIP programmed for proteolysis.

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12 December 2020

Identification of genes that modify ataxin-1-induced neurodegeneration.

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12 December 2020

Impairment of the ubiquitin-proteasome system by protein aggregation

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12 December 2020

Cleavage of BID by caspase 8 mediates the mitochondrial damage in the Fas pathway of apoptosis

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12 December 2020

Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

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12 December 2020

Chaperones and aging: role in neurodegeneration and in other civilizational diseases

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12 December 2020

Support for the prion hypothesis for inheritance of a phenotypic trait in yeast

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12 December 2020

Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line

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12 December 2020

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

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12 December 2020

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

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12 December 2020

A one-hit model of cell death in inherited neuronal degenerations

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12 December 2020

Expansion of polyglutamine induces the formation of quasi-aggregate in the early stage of protein fibrillization

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12 December 2020

Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression

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12 December 2020

HSP27 inhibits cytochrome c-dependent activation of procaspase-9.

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12 December 2020

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

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12 December 2020

Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract

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12 December 2020

Down-regulation of heat shock protein 27 in neuronal cells and non-neuronal cells expressing mutant ataxin-3

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12 December 2020

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

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12 December 2020

PKB binding proteins. Getting in on the Akt.

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12 December 2020

FLICE, a novel FADD-homologous ICE/CED-3-like protease, is recruited to the CD95 (Fas/APO-1) death--inducing signaling complex

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12 December 2020

The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

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12 December 2020

The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

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12 December 2020

Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function.

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12 December 2020

Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders

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12 December 2020

Identification of ter94, Drosophila VCP, as a modulator of polyglutamine-induced neurodegeneration

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12 December 2020

S-thiolation of HSP27 regulates its multimeric aggregate size independently of phosphorylation

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12 December 2020

Amyloid fibrillogenesis: themes and variations

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12 December 2020

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

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12 December 2020

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

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12 December 2020

Polyglutamine fibrillogenesis: the pathway unfolds

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12 December 2020

A molecular chaperone complex at the lysosomal membrane is required for protein translocation

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12 December 2020

Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease

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12 December 2020

Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease

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12 December 2020

Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease

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12 December 2020

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

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12 December 2020

Protein-damaging stresses activate c-Jun N-terminal kinase via inhibition of its dephosphorylation: a novel pathway controlled by HSP72.

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12 December 2020

Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7

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12 December 2020

Molecular chaperones in the regulation of signal transduction

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12 December 2020

The localization and interactions of huntingtin

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12 December 2020

Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

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12 December 2020

Posttranslational quality control: folding, refolding, and degrading proteins.

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12 December 2020

Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

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12 December 2020

Purification of polyglutamine aggregates and identification of elongation factor-1alpha and heat shock protein 84 as aggregate-interacting proteins.

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12 December 2020

Inhibition of superoxide generation from neuronal nitric oxide synthase by heat shock protein 90: implications in NOS regulation

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12 December 2020

Glycogen synthase kinase-3beta inhibitors prevent cellular polyglutamine toxicity caused by the Huntington's disease mutation

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12 December 2020

Hsp27 upregulation and phosphorylation is required for injured sensory and motor neuron survival

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12 December 2020

Large unphosphorylated aggregates as the active form of hsp27 which controls intracellular reactive oxygen species and glutathione levels and generates a protection against TNFalpha in NIH-3T3-ras cells

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12 December 2020

Polyglutamine aggregates stimulate ER stress signals and caspase-12 activation

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12 December 2020

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

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12 December 2020

Molecular chaperones in the cytosol: from nascent chain to folded protein

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12 December 2020

Wild-type huntingtin protects from apoptosis upstream of caspase-3.

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12 December 2020

Modulation of polyglutamine-induced cell death by genes identified by expression profiling

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12 December 2020

Autophagy is activated by apoptotic signalling in sympathetic neurons: an alternative mechanism of death execution

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12 December 2020

The chaperone function of hsp70 is required for protection against stress-induced apoptosis

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12 December 2020

A molecular investigation of true dominance in Huntington's disease

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12 December 2020

Of molecular interactions, mice and mechanisms: new insights into Huntington's disease

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12 December 2020

Harm's way: polyglutamine repeats and the activation of an apoptotic pathway.

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12 December 2020

Triggering of neuronal cell death by accumulation of activated SEK1 on nuclear polyglutamine aggregations in PML bodies

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12 December 2020

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons.

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12 December 2020

Heat-shock protein 70 antagonizes apoptosis-inducing factor

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12 December 2020

The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human.

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12 December 2020

Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production

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12 December 2020

Apoptotic pathways: the roads to ruin

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12 December 2020

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

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12 December 2020

Hsp27 functions as a negative regulator of cytochrome c-dependent activation of procaspase-3.

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12 December 2020

Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein

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12 December 2020

Induction of 27-kDa heat shock protein following cerebral ischemia in a rat model of ischemic tolerance

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12 December 2020

Heat shock protein 70 binding inhibits the nuclear import of apoptosis-inducing factor.

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12 December 2020

Small heat-shock proteins and their potential role in human disease

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12 December 2020

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

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12 December 2020

Heat Shock Protein 27 Controls Apoptosis by Regulating Akt Activation

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12 December 2020

Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models.

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12 December 2020

Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

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12 December 2020

Time course of polyglutamine aggregate body formation and cell death: enhanced growth in nucleus and an interval for cell death

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12 December 2020

Activation of protein kinase B (Akt/RAC-protein kinase) by cellular stress and its association with heat shock protein Hsp27.

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12 December 2020

Protection of neuronal cells from apoptosis by Hsp27 delivered with a herpes simplex virus-based vector

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12 December 2020

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

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12 December 2020

Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera

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12 December 2020

Ubiquitylation of BAG-1 suggests a novel regulatory mechanism during the sorting of chaperone substrates to the proteasome

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12 December 2020

Huntingtin localization in brains of normal and Huntington's disease patients

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12 December 2020

Caspase-independent cell deaths

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12 December 2020

Cooperation of a ubiquitin domain protein and an E3 ubiquitin ligase during chaperone/proteasome coupling

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12 December 2020

Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi

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12 December 2020

Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila

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12 December 2020

Negative regulation of the Apaf-1 apoptosome by Hsp70

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12 December 2020

The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins

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12 December 2020

Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation

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12 December 2020

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

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12 December 2020

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

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12 December 2020

Modification of brain aging and neurodegenerative disorders by genes, diet, and behavior

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12 December 2020

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

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12 December 2020

The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis

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12 December 2020

Regulation of Aging and Age-Related Disease by DAF-16 and Heat-Shock Factor

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12 December 2020

Fourteen and counting: unraveling trinucleotide repeat diseases

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12 December 2020

Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.

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12 December 2020

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

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12 December 2020

HSP27 is a ubiquitin-binding protein involved in I-kappaBalpha proteasomal degradation

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12 December 2020

Kennedy's disease. Phosphorylation of the polyglutamine-expanded form of androgen receptor regulates its cleavage by caspase-3 and enhances cell death

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12 December 2020

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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12 December 2020

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

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12 December 2020

Endonuclease G is an apoptotic DNase when released from mitochondria

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12 December 2020

Pro-apoptotic protein kinase C delta is associated with intranuclear inclusions in a transgenic model of Huntington's disease.

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12 December 2020

Aggregation of proteins with expanded glutamine and alanine repeats of the glutamine-rich and asparagine-rich domains of Sup35 and of the amyloid beta-peptide of amyloid plaques.

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12 December 2020

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

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12 December 2020

Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease

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12 December 2020

ASK1 is required for sustained activations of JNK/p38 MAP kinases and apoptosis

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12 December 2020

Hsp72-mediated suppression of c-Jun N-terminal kinase is implicated in development of tolerance to caspase-independent cell death

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12 December 2020

Cytochrome C and caspase-9 expression in Huntington's disease

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12 December 2020

Upregulation of HSP27 in a Transgenic Model of ALS

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12 December 2020

Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity

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12 December 2020

The heat-shock response: regulation and function of heat-shock proteins and molecular chaperones.

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12 December 2020

Suppression of stress kinase JNK is involved in HSP72-mediated protection of myogenic cells from transient energy deprivation. HSP72 alleviates the stewss-induced inhibition of JNK dephosphorylation

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12 December 2020

Autophagy in the eukaryotic cell

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12 December 2020

Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease

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12 December 2020

Trinucleotide repeats: mechanisms and pathophysiology

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12 December 2020

Signalling for survival and death in neurones: the role of stress-activated kinases, JNK and p38

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12 December 2020

Protein tyrosine phosphatases are up-regulated and participate in cell death induced by polyglutamine expansion

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12 December 2020

Molecular characterization of mitochondrial apoptosis-inducing factor

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12 December 2020

Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases

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12 December 2020

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

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12 December 2020

3pK, a novel mitogen-activated protein (MAP) kinase-activated protein kinase, is targeted by three MAP kinase pathways

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12 December 2020

Hsp27 negatively regulates cell death by interacting with cytochrome c.

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12 December 2020

Hsp60 accelerates the maturation of pro-caspase-3 by upstream activator proteases during apoptosis

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12 December 2020

Death receptors: signaling and modulation

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12 December 2020

Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila.

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12 December 2020

Identifiers

DOI

10.1385/JMN:23:1-2:069

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Dimensions Publication ID

10 August 2017

1037358252

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