The role of HSP70 and its co-chaperones in protein misfolding, aggregation and disease. (Q38285309)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

review article

1 reference

title

The role of HSP70 and its co-chaperones in protein misfolding, aggregation and disease (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

Emma J Duncan

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

Jacqueline van der Spuy

series ordinal

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

publication date

1 January 2015

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

Sub-cellular biochemistry

24 February 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

volume

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

page(s)

243-273

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model.

24 February 2020

cites work

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Comparison of lymphoblast mitochondria from normal subjects and patients with Barth syndrome using electron microscopic tomography

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The sacsin repeating region (SRR): a novel Hsp90-related supra-domain associated with neurodegeneration

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The gene disrupted in Marinesco-Sjögren syndrome encodes SIL1, an HSPA5 cochaperone

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Novel SIL1 mutations and exclusion of functional candidate genes in Marinesco-Sjögren syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Hsp90 inhibition protects against inherited retinal degeneration

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Roles of molecular chaperones in protein misfolding diseases

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A novel X-linked gene, G4.5. is responsible for Barth syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A rare recessive distal hereditary motor neuropathy with HSJ1 chaperone mutation

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Chaperoning brain degeneration

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Autosomal recessive spastic ataxia of Charlevoix–Saguenay: An overview

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: implications for Barth Syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The Hsp70 and Hsp60 chaperone machines

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A chaperone pathway in protein disaggregation. Hsp26 alters the nature of protein aggregates to facilitate reactivation by Hsp104.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Decreased protein synthesis of Hsp27 associated with cellular toxicity in a cell model of Machado-Joseph disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The Chaperone Environment at the Cytoplasmic Face of the Endoplasmic Reticulum Can Modulate Rhodopsin Processing and Inclusion Formation

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Neuronal DnaJ proteins HSJ1a and HSJ1b: a role in linking the Hsp70 chaperone machine to the ubiquitin-proteasome system?

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structure, function and evolution of DnaJ: conservation and adaptation of chaperone function

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Co-chaperone CHIP promotes aggregation of ataxin-1.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

BAP, a mammalian BiP-associated protein, is a nucleotide exchange factor that regulates the ATPase activity of BiP.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Exome sequencing identifies a DNAJB6 mutation in a family with dominantly-inherited limb-girdle muscular dystrophy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Autophagy: in sickness and in health

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Impaired degradation of mutant alpha-synuclein by chaperone-mediated autophagy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutation of DNAJC19, a human homologue of yeast inner mitochondrial membrane co-chaperones, causes DCMA syndrome, a novel autosomal recessive Barth syndrome-like condition

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Protein folding and misfolding

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

J protein cochaperone of the mitochondrial inner membrane required for protein import into the mitochondrial matrix

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Dual role of the mitochondrial chaperone Mdj1p in inheritance of mitochondrial DNA in yeast

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Cardiolipin deficiency affects respiratory chain function and organization in an induced pluripotent stem cell model of Barth syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Binding of non-native protein to Hsp25 during heat shock creates a reservoir of folding intermediates for reactivation.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

ARSACS, a spastic ataxia common in northeastern Québec, is caused by mutations in a new gene encoding an 11.5-kb ORF

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Identification of a new homozygous frameshift insertion mutation in the SIL1 gene in 3 Japanese patients with Marinesco-Sjögren syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Inhibiting Drp1-mediated mitochondrial fission selectively prevents the release of cytochrome c during apoptosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Phenotype-genotype correlations in patients with Marinesco-Sjögren syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Co-Chaperone HSJ1a Dually Regulates the Proteasomal Degradation of Ataxin-3

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mitochondrial cardiolipin involved in outer-membrane protein biogenesis: implications for Barth syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

HEPN: a common domain in bacterial drug resistance and human neurodegenerative proteins

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Bimoclomol, a heat shock protein co-inducer, acts by the prolonged activation of heat shock factor-1

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Disassembling protein aggregates in the yeast cytosol. The cooperation of Hsp26 with Ssa1 and Hsp104.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A crucial role of mitochondrial Hsp40 in preventing dilated cardiomyopathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Tafazzin knockdown causes hypertrophy of neonatal ventricular myocytes

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutations in the HSP27 (HSPB1) gene cause dominant, recessive, and sporadic distal HMN/CMT type 2

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

C-terminal mutations destabilize SIL1/BAP and can cause Marinesco-Sjögren syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The Pam18/Tim14-Pam16/Tim16 complex of the mitochondrial translocation motor: the formation of a stable complex from marginally stable proteins

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutant HSPB8 causes motor neuron-specific neurite degeneration.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structural basis of interdomain communication in the Hsc70 chaperone.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structure of the XPC binding domain of hHR23A reveals hydrophobic patches for protein interaction

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Guidelines for the nomenclature of the human heat shock proteins

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A novel mutation in BAP/SIL1 gene causes Marinesco-Sjögren syndrome in an extended pedigree.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutations in MKKS cause obesity, retinal dystrophy and renal malformations associated with Bardet-Biedl syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Genetic suppression of polyglutamine toxicity in Drosophila

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Small heat shock protein 27 mutation in a Japanese patient with distal hereditary motor neuropathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Polyglutamine protein aggregates are dynamic

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

MKKS/BBS6, a divergent chaperonin-like protein linked to the obesity disorder Bardet-Biedl syndrome, is a novel centrosomal component required for cytokinesis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Update on Hsp90 inhibitors in clinical trial.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Protein folding and quality control in the endoplasmic reticulum

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

AIPL1, a protein associated with childhood blindness, interacts with alpha-subunit of rod phosphodiesterase (PDE6) and is essential for its proper assembly

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutant small heat shock protein B3 causes motor neuropathy: utility of a candidate gene approach

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structural Basis of Defects in the Sacsin HEPN Domain Responsible for Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Formation and toxicity of soluble polyglutamine oligomers in living cells

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Arimoclomol: a potential therapy under development for ALS.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structure and function of Hip, an attenuator of the Hsp70 chaperone cycle

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Autosomal dominant congenital cataract associated with a missense mutation in the human alpha crystallin gene CRYAA

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Tid1, a cochaperone of the heat shock 70 protein and the mammalian counterpart of the Drosophila tumor suppressor l(2)tid, is critical for early embryonic development and cell survival

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mitochondrial dynamics and neurodegeneration

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Cell death triggered by a novel mutation in the alphaA-crystallin gene underlies autosomal dominant cataract linked to chromosome 21q

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A missense mutation in Tbce causes progressive motor neuronopathy in mice

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Autosomal recessive spastic ataxia of Charlevoix-Saguenay: a report of MR imaging in 5 patients

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Novel mutations in the TAZ gene in patients with Barth syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

CREB-binding protein sequestration by expanded polyglutamine

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structure and function of Tim14 and Tim16, the J and J-like components of the mitochondrial protein import motor

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Modulation of neurodegeneration by molecular chaperones

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Solution structures of UBA domains reveal a conserved hydrophobic surface for protein-protein interactions

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Essential role of TID1 in maintaining mitochondrial membrane potential homogeneity and mitochondrial DNA integrity

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Genome-wide studies of copy number variation and exome sequencing identify rare variants in BAG3 as a cause of dilated cardiomyopathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The heat-shock response co-inducer arimoclomol protects against retinal degeneration in rhodopsin retinitis pigmentosa.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutation of TBCE causes hypoparathyroidism-retardation-dysmorphism and autosomal recessive Kenny-Caffey syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Structure and mechanism of the Hsp90 molecular chaperone machinery

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Discovery and development of Hsp90 inhibitors: a promising pathway for cancer therapy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Comparative analysis and functional mapping of SACS mutations reveal novel insights into sacsin repeated architecture.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Opinion: What is the role of protein aggregation in neurodegeneration?

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Modulation of polyglutamine inclusion formation by the Hsp70 chaperone machine.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The Ubiquitin-Proteasome System in Huntington's Disease: Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Methylation-controlled J-protein MCJ acts in the import of proteins into human mitochondria

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Positional cloning of the gene for X-linked retinitis pigmentosa 2

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Arl3 and RP2 mediated assembly and traffic of membrane associated cilia proteins.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free system

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Unraveling the intricate organization of mammalian mitochondrial presequence translocases: existence of multiple translocases for maintenance of mitochondrial function.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutations in a new photoreceptor-pineal gene on 17p cause Leber congenital amaurosis

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Heat shock proteins as emerging therapeutic targets

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutation of a gene encoding a putative chaperonin causes McKusick-Kaufman syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS): expanding the genetic, clinical and imaging spectrum

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Novel mutations in the SIL1 gene in a Japanese pedigree with the Marinesco-Sjögren syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Mutation analysis of the small heat shock protein 27 gene in chinese patients with Charcot-Marie-Tooth disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Small heat-shock protein 22 mutated in autosomal dominant Charcot-Marie-Tooth disease type 2L

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Toxic proteins in neurodegenerative disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Characterization of the human heart mitochondrial proteome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

ARSACS in the Dutch population: a frequent cause of early-onset cerebellar ataxia

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

A missense mutation in the alphaB-crystallin chaperone gene causes a desmin-related myopathy

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Enantioselective plasma protein binding of bimoclomol

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

HSPB7 is the most potent polyQ aggregation suppressor within the HSPB family of molecular chaperones

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

HSJ1 is a neuronal shuttling factor for the sorting of chaperone clients to the proteasome

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis.

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

The enzymatic function of tafazzin

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation

31 March 2020

1 reference

https://api.crossref.org/works/10.1007%2F978-3-319-11731-7_12

10.1007/978-3-319-11731-7_12

31 March 2020

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25487025%20AND%20SRC:MED&resulttype=core&format=json