Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants (Q41969514)

14 October 2017

Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants (English)

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amyotrophic lateral sclerosis

14 October 2017

1 reference

Cami K Bruns

1

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14 October 2017

Ron R Kopito

series ordinal

2

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14 October 2017

language of work or name

English

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publication date

25 January 2007

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14 October 2017

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14 October 2017

26

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14 October 2017

3

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14 October 2017

855-866

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describes a project that uses

14 October 2017

ImageQuant

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC1794386/fullTextXML

inferred from PubMed Central ID database lookup

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC1794386/fullTextXML

inferred from PubMed Central ID database lookup

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Activation of superoxide dismutases: putting the metal to the pedal

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Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis.

5 June 2018

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Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis.

5 June 2018

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Systematically perturbed folding patterns of amyotrophic lateral sclerosis (ALS)-associated SOD1 mutants

5 June 2018

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

5 June 2018

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Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg.

5 June 2018

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Structural basis of protein kinetic stability: resistance to sodium dodecyl sulfate suggests a central role for rigidity and a bias toward beta-sheet structure.

5 June 2018

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Oxygen-induced maturation of SOD1: a key role for disulfide formation by the copper chaperone CCS.

5 June 2018

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Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis

5 June 2018

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Solution structure of Apo Cu,Zn superoxide dismutase: role of metal ions in protein folding

5 June 2018

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ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

5 June 2018

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Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro

5 June 2018

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Is there a unifying mechanism for protein folding?

5 June 2018

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Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis.

5 June 2018

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Heterodimeric structure of superoxide dismutase in complex with its metallochaperone

5 June 2018

1 reference

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Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.

5 June 2018

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Protein folding in vivo: the importance of molecular chaperones

5 June 2018

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Transition-state structure as a unifying basis in protein-folding mechanisms: contact order, chain topology, stability, and the extended nucleus mechanism

5 June 2018

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Undetectable intracellular free copper: the requirement of a copper chaperone for superoxide dismutase

5 June 2018

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The fundamentals of protein folding: bringing together theory and experiment

5 June 2018

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Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis

5 June 2018

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Expression of the copper-zinc superoxide dismutase gene in amyotrophic lateral sclerosis

5 June 2018

1 reference

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ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

5 June 2018

1 reference

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Principles of chaperone-assisted protein folding: differences between in vitro and in vivo mechanisms

5 June 2018

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Aurintricarboxylic acid: inhibitor of initiation of protein synthesis

5 June 2018

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On the stability of bovine superoxide dismutase. The effects of metals.

5 June 2018

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Gene expression using cell-free systems

5 June 2018

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Trigger factor forms a protective shield for nascent polypeptides at the ribosome.

5 June 2018

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Mechanism and thermodynamics of guanidinium chloride-induced denaturation of ALS-associated mutant Cu,Zn superoxide dismutases

18 August 2018

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Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced form

18 August 2018

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Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

18 August 2018

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Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature

18 August 2018

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Familial amyotrophic lateral sclerosis-associated mutations decrease the thermal stability of distinctly metallated species of human copper/zinc superoxide dismutase

18 August 2018

1 reference

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Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues

18 August 2018

1 reference

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Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1794386

Amyotrophic lateral sclerosis associated with genetic abnormalities in the gene encoding Cu/Zn superoxide dismutase: molecular pathology of five new cases, and comparison with previous reports and 73 sporadic cases of ALS.

18 August 2018

1 reference

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Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement.

18 August 2018

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1794386

18 August 2018

https://api.crossref.org/works/10.1038%2FSJ.EMBOJ.7601528

7 January 2021

inferred from DOI database lookup

Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1794386

?True? sporadic ALS associated with a novel SOD-1 mutation

3 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1794386

Accumulation of neurofilaments and SOD1-immunoreactive products in a patient with familial amyotrophic lateral sclerosis with I113T SOD1 mutation

2 December 2018

1 reference

12 December 2020

Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation: immunohistochemical and immunoelectron microscopical analyses

1 reference

12 December 2020

Erratum: Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

1 reference

12 December 2020

Folding of nascent polypeptide chains in a high molecular mass assembly with molecular chaperones

1 reference

12 December 2020

A spectroscopic characterization of a monomeric analog of copper, zinc superoxide dismutase

1 reference

12 December 2020

Superoxide dismutase concentration and activity in familial amyotrophic lateral sclerosis

1 reference

12 December 2020

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

1 reference

12 December 2020

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

1 reference

12 December 2020

Presence of Cu/Zn superoxide dismutase (SOD) immunoreactivity in neuronal hyaline inclusions in spinal cords from mice carrying a transgene for Gly93Ala mutant human Cu/Zn SOD

1 reference

12 December 2020

Clustering of ALS patients in central Italy due to the occurrence of the L84F SOD1 gene mutation

1 reference

12 December 2020

Identifiers

DOI

10.1038/SJ.EMBOJ.7601528

1 reference

14 October 2017

1 reference

14 October 2017

PubMed publication ID

17255946

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