(Q34348757)

1 August 2017

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title

Unfolding the role of chaperones and chaperonins in human disease (English)

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1 August 2017

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Slavotinek AM

1

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1 August 2017

Biesecker LG

series ordinal

2

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1 August 2017

publication date

1 September 2001

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1 August 2017

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1 August 2017

volume

17

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1 August 2017

issue

9

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1 August 2017

page(s)

528-535

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Principles that govern the folding of protein chains

1 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Principles of protein folding in the cellular environment

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Effects of macromolecular crowding on protein folding and aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Recombination of protein domains facilitated by co-translational folding in eukaryotes

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Protein folding in vivo: the importance of molecular chaperones

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

1 reference

7 January 2021

Chaperone-mediated protein folding

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Roles of molecular chaperones in cytoplasmic protein folding

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The Hsp70 and Hsp60 Chaperone Machines

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Substrate shuttling between the DnaK and GroEL systems indicates a chaperone network promoting protein folding.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Molecular chaperones and the art of recognizing a lost cause

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Initial sequencing and analysis of the human genome

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

GroEL/GroES: structure and function of a two-stroke folding machine

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

GroEL-GroES cycling: ATP and nonnative polypeptide direct alternation of folding-active rings

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Chaperonin function: folding by forced unfolding

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Identification of in vivo substrates of the chaperonin GroEL

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The chaperonin containing t-complex polypeptide 1 (TCP-1). Multisubunit machinery assisting in protein folding and assembly in the eukaryotic cytosol.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Crystal structure of the thermosome, the archaeal chaperonin and homolog of CCT

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The thermosome: chaperonin with a built-in lid

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Eukaryotic type II chaperonin CCT interacts with actin through specific subunits

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Crystal structure of the beta-apical domain of the thermosome reveals structural plasticity in the protrusion region

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Mutations in MKKS cause Bardet-Biedl syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Genetic and mutational analyses of a large multiethnic Bardet-Biedl cohort reveal a minor involvement of BBS6 and delineate the critical intervals of other loci

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Positional cloning of a novel gene on chromosome 16q causing Bardet-Biedl syndrome (BBS2)

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Identification of the gene that, when mutated, causes the human obesity syndrome BBS4.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The role of DnaJ-like proteins in glucocorticoid receptor.hsp90 heterocomplex assembly by the reconstituted hsp90.p60.hsp70 foldosome complex.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Site-directed mutations within the core "alpha-crystallin" domain of the small heat-shock protein, human alphaB-crystallin, decrease molecular chaperone functions

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Structural and functional consequences of the mutation of a conserved arginine residue in alphaA and alphaB crystallins

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Lens epithelial cells derived from alphaB-crystallin knockout mice demonstrate hyperproliferation and genomic instability

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Missense mutations in desmin associated with familial cardiac and skeletal myopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Protein precipitation: a common etiology in neurodegenerative disorders?

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Genetic suppression of polyglutamine toxicity in Drosophila

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Distinct molecular mechanisms and divergent endocytotic pathways of AMPA receptor internalization

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Presenilin mutants subvert chaperone function.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The chaperone BiP/GRP78 binds to amyloid precursor protein and decreases Abeta40 and Abeta42 secretion.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Defective protein folding as a basis of human disease

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The Molecular Chaperone Hsc70 Assists the in VitroFolding of the N-terminal Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Expression analysis of phenylketonuria mutations. Effect on folding and stability of the phenylalanine hydroxylase protein

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Effects of two mutations detected in medium chain acyl-CoA dehydrogenase (MCAD)-deficient patients on folding, oligomer assembly, and stability of MCAD enzyme

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

The molecular basis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in compound heterozygous patients: is there correlation between genotype and phenotype?

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Ethylmalonic aciduria is associated with an amino acid variant of short chain acyl-coenzyme A dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

Hsp90 & Co. - a holding for folding

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2801%2902413-1

7 January 2021

10.1016/S0168-9525(01)02413-1

Identifiers

DOI

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1 August 2017

1 reference