(Q47889576)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

review article

0 references

title

The Role of Metal Binding in the Amyotrophic Lateral Sclerosis-Related Aggregation of Copper-Zinc Superoxide Dismutase (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

amyotrophic lateral sclerosis

28 March 2020

main subject

3 references

25 April 2019

28 September 2019

4 November 2019

1 reference

28 September 2019

1 reference

4 November 2019

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

author name string

Ivana Sirangelo

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

language of work or name

English

0 references

publication date

29 August 2017

4 references

25 April 2019

28 September 2019

4 November 2019

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

4 references

25 April 2019

28 September 2019

4 November 2019

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

volume

22

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

issue

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

Creative Commons Attribution

28 March 2020

copyright license

3 references

25 April 2019

28 September 2019

4 November 2019

0 references

on focus list of Wikimedia project

ScienceSource

0 references

cites work

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Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

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Atomic structure and hierarchical assembly of a cross- amyloid fibril

21 January 2018

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Copper homeostasis and neurodegenerative disorders (Alzheimer's, prion, and Parkinson's diseases and amyotrophic lateral sclerosis).

21 January 2018

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Misfolded proteins in Alzheimer's disease and type II diabetes.

21 January 2018

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Membrane disruption and early events in the aggregation of the diabetes related peptide IAPP from a molecular perspective

21 January 2018

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Development of bifunctional stilbene derivatives for targeting and modulating metal-amyloid-β species.

21 January 2018

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A two-site mechanism for the inhibition of IAPP amyloidogenesis by zinc

21 January 2018

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Design of small molecules that target metal-A{beta} species and regulate metal-induced A{beta} aggregation and neurotoxicity.

21 January 2018

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

21 January 2018

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Activation of superoxide dismutases: putting the metal to the pedal

21 January 2018

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SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization

21 January 2018

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Amyotrophic lateral sclerosis

21 January 2018

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Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis?

21 January 2018

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Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis.

21 January 2018

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Aggregation of copper-zinc superoxide dismutase in familial and sporadic ALS

21 January 2018

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An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

21 January 2018

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Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis

21 January 2018

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Pathological roles of wild-type cu, zn-superoxide dismutase in amyotrophic lateral sclerosis.

21 January 2018

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An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

21 January 2018

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Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: a possible general mechanism for familial ALS

21 January 2018

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Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase

21 January 2018

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https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis

21 January 2018

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https://api.crossref.org/works/10.3390%2FMOLECULES22091429

SOD1 aggregation and ALS: role of metallation states and disulfide status

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Glycation in Demetalated Superoxide Dismutase 1 Prevents Amyloid Aggregation and Produces Cytotoxic Ages Adducts

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Relationship between mutant Cu/Zn superoxide dismutase 1 maturation and inclusion formation in cell models

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site

21 January 2018

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https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Structural and dynamic aspects related to oligomerization of apo SOD1 and its mutants

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

How do ALS-associated mutations in superoxide dismutase 1 promote aggregation of the protein?

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Insights into SOD1-linked amyotrophic lateral sclerosis from NMR studies of Ni(2+)- and other metal-ion-substituted wild-type copper-zinc superoxide dismutases

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Mitochondrial dysfunction in amyotrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Conserved patterns in the Cu,Zn superoxide dismutase family.

21 January 2018

1 reference

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Superoxide dismutase folding/unfolding pathway: role of the metal ions in modulating structural and dynamical features.

21 January 2018

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Mechanisms of the copper-dependent turnover of the copper chaperone for superoxide dismutase

21 January 2018

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In-cell NMR in E. coli to monitor maturation steps of hSOD1.

21 January 2018

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Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS)

21 January 2018

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

21 January 2018

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Mechanisms for activating Cu- and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone

21 January 2018

1 reference

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Dissociation of human copper-zinc superoxide dismutase dimers using chaotrope and reductant. Insights into the molecular basis for dimer stability

21 January 2018

1 reference

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Folding of human superoxide dismutase: disulfide reduction prevents dimerization and produces marginally stable monomers

21 January 2018

1 reference

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Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Aberrant zinc binding to immature conformers of metal-free copper-zinc superoxide dismutase triggers amorphous aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Heterodimeric structure of superoxide dismutase in complex with its metallochaperone

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Structure and dynamics of copper-free SOD: The protein before binding copper

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

pH-dependent migration of copper(II) to the vacant zinc-binding site of zinc-free bovine erythrocyte superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Visualization of redox-controlled protein fold in living cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Calorimetric analysis of thermodynamic stability and aggregation for apo and holo amyotrophic lateral sclerosis-associated Gly-93 mutants of superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Unraveling the mechanisms involved in motor neuron degeneration in ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Amyotrophic lateral sclerosis associated with mutations in the CuZn superoxide dismutase gene

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

ALS: a disease of motor neurons and their nonneuronal neighbors

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Cellular toxicity of mutant SOD1 protein is linked to an easily soluble, non-aggregated form in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Direct Conversion of an Enzyme from Native-like to Amyloid-like Aggregates within Inclusion Bodies.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

SOD1 exhibits allosteric frustration to facilitate metal binding affinity

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Transient structural distortion of metal-free Cu/Zn superoxide dismutase triggers aberrant oligomerization

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Kinetic stability of Cu/Zn superoxide dismutase is dependent on its metal ligands: implications for ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Folding catalysis by transient coordination of Zn2+ to the Cu ligands of the ALS-associated enzyme Cu/Zn superoxide dismutase 1

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Zinc binding modulates the entire folding free energy surface of human Cu,Zn superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Local unfolding in a destabilized, pathogenic variant of superoxide dismutase 1 observed with H/D exchange and mass spectrometry

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Metal-free superoxide dismutase-1 and three different amyotrophic lateral sclerosis variants share a similar partially unfolded beta-barrel at physiological temperature

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Local unfolding of Cu, Zn superoxide dismutase monomer determines the morphology of fibrillar aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Mechanisms of biosynthesis of mammalian copper/zinc superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Calcium ions promote superoxide dismutase 1 (SOD1) aggregation into non-fibrillar amyloid: a link to toxic effects of calcium overload in amyotrophic lateral sclerosis (ALS)?

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Calcium signalling: dynamics, homeostasis and remodelling

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Structural and Biophysical Properties of the Pathogenic SOD1 Variant H46R/H48Q †

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Rationalization of the effects of mutations on peptide and protein aggregation rates.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Copper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 Mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Metal ions as modulators of protein conformation and misfolding in neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Prediction of the Absolute Aggregation Rates of Amyloidogenic Polypeptide Chains

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FMOLECULES22091429

Penicillamine in amyotrophic lateral sclerosis

21 January 2018

1 reference

12 December 2020

Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite

1 reference

12 December 2020

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

1 reference

12 December 2020

Metals in spinal cord tissue of patients dying of motor neuron disease

1 reference

12 December 2020

The unusually stable quaternary structure of human Cu,Zn-superoxide dismutase 1 is controlled by both metal occupancy and disulfide status

1 reference

12 December 2020

The coupling between disulphide status, metallation and dimer interface strength in Cu/Zn superoxide dismutase

1 reference

12 December 2020

The metal binding properties of the zinc site of yeast copper-zinc superoxide dismutase: implications for amyotrophic lateral sclerosis

1 reference

12 December 2020

Binding of a single zinc ion to one subunit of copper-zinc superoxide dismutase apoprotein substantially influences the structure and stability of the entire homodimeric protein

1 reference

12 December 2020

10.3390/MOLECULES22091429

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28850080%20AND%20SRC:MED&resulttype=core&format=json

28 March 2020

1 reference