The role of molecular chaperones in human misfolding diseases (Q37459598)

20 August 2017

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title

The role of molecular chaperones in human misfolding diseases (English)

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20 August 2017

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2

F Ulrich Hartl

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20 August 2017

Sarah A Broadley

1

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PubMed ID

19393652

20 August 2017

language of work or name

English

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publication date

23 April 2009

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20 August 2017

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20 August 2017

583

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20 August 2017

16

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20 August 2017

2647-2653

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

20 August 2017

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Molecular chaperones and protein quality control

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Protein misfolding, functional amyloid, and human disease

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

N-terminal polyglutamine-containing fragments inhibit androgen receptor transactivation function

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Competing pathways determine fibril morphology in the self-assembly of beta2-microglobulin into amyloid.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock protein 70 inhibits alpha-synuclein fibril formation via preferential binding to prefibrillar species

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Modulation of neurodegeneration by molecular chaperones

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Contribution of somal Lewy bodies to neuronal death.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Pharmacological prevention of Parkinson disease in Drosophila.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Clinical, pathological, and neurochemical changes in dementia: A subgroup with preserved mental status and numerous neocortical plaques

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Polyglutamine neurodegeneration: protein misfolding revisited.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Zeroing in on the pathogenic form of alpha-synuclein and its mechanism of neurotoxicity in Parkinson's disease

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Adapting proteostasis for disease intervention

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Hsp70 chaperones: cellular functions and molecular mechanism

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The mechanism of Hsp70 chaperones: (entropic) pulling the models together

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The co-chaperone carboxyl terminus of Hsp70-interacting protein (CHIP) mediates alpha-synuclein degradation decisions between proteasomal and lysosomal pathways

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Characterization of Hsp70 binding and nucleotide exchange by the yeast Hsp110 chaperone Sse1.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Structural basis for the cooperation of Hsp70 and Hsp110 chaperones in protein folding

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Mutations in SIL1 cause Marinesco-Sjögren syndrome, a cerebellar ataxia with cataract and myopathy

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Roles of molecular chaperones in protein misfolding diseases

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Progressive disruption of cellular protein folding in models of polyglutamine diseases.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Neuronal cells show regulatory differences in the hsp70 gene response.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Gene regulation and DNA damage in the ageing human brain

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Regulation of heat shock gene transcription in neuronal cells

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Opposing activities protect against age-onset proteotoxicity

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Decreased cAMP response element-mediated transcription: an early event in exon 1 and full-length cell models of Huntington's disease that contributes to polyglutamine pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

alpha-Synuclein forms a complex with transcription factor Elk-1.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Transcriptional abnormalities in Huntington disease

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Novel therapeutic strategies for the treatment of protein-misfolding diseases

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock protein 70 participates in the neuroprotective response to intracellularly expressed beta-amyloid in neurons.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock treatment reduces beta amyloid toxicity in vivo by diminishing oligomers

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Chaperones increase association of tau protein with microtubules

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Hsp70 Reduces alpha-Synuclein Aggregation and Toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Association of Bcl-2 with misfolded prion protein is linked to the toxic potential of cytosolic PrP.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Hsp40 molecules that target to the ubiquitin-proteasome system decrease inclusion formation in models of polyglutamine disease

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

CHIP targets toxic alpha-Synuclein oligomers for degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Co-chaperone CHIP promotes aggregation of ataxin-1.

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

CHIP and HSPs interact with beta-APP in a proteasome-dependent manner and influence Abeta metabolism.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

CHIP-Hsc70 Complex Ubiquitinates Phosphorylated Tau and Enhances Cell Survival

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

BAG-1 associates with the polyglutamine-expanded huntingtin aggregates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock proteins 70 and 90 inhibit early stages of amyloid beta-(1-42) aggregation in vitro.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Interactions between Hsp70 and the hydrophobic core of alpha-synuclein inhibit fibril assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Mitochondrial hsp60 chaperonopathy causes an autosomal-recessive neurodegenerative disorder linked to brain hypomyelination and leukodystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Mechanism of the eukaryotic chaperonin: protein folding in the chamber of secrets.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Systems analyses reveal two chaperone networks with distinct functions in eukaryotic cells

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Cystosolic chaperonin subunits have a conserved ATPase domain but diverged polypeptide-binding domains.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Defining the TRiC/CCT interactome links chaperonin function to stabilization of newly made proteins with complex topologies

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

In vivo newly translated polypeptides are sequestered in a protected folding environment

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The cytosolic class II chaperonin CCT recognizes delineated hydrophobic sequences in its target proteins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

The CCT chaperonin promotes activation of the anaphase-promoting complex through the generation of functional Cdc20.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Tumorigenic mutations in VHL disrupt folding in vivo by interfering with chaperonin binding

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Cytosolic chaperonin protects folding intermediates of Gbeta from aggregation by recognizing hydrophobic beta-strands

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock response modulators as therapeutic tools for diseases of protein conformation

21 January 2018

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https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Phase II trial of 17-allylamino-17-demethoxygeldanamycin in patients with metastatic melanoma.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

A phase II trial of 17-(Allylamino)-17-demethoxygeldanamycin in patients with papillary and clear cell renal cell carcinoma.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

A non-toxic Hsp90 inhibitor protects neurons from Abeta-induced toxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Sirtuins--novel therapeutic targets to treat age-associated diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1 mouse model of ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Celastrols as inducers of the heat shock response and cytoprotection.

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

Intraneuronal -Amyloid Expression Downregulates the Akt Survival Pathway and Blunts the Stress Response

21 January 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.FEBSLET.2009.04.029

10.1016/J.FEBSLET.2009.04.029

21 January 2018

Identifiers

DOI

1 reference

20 August 2017

1 reference

PubMed ID

19393652