Nonamyloid aggregates arising from mature copper/zinc superoxide dismutases resemble those observed in amyotrophic lateral sclerosis (Q34438898)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

title

Nonamyloid aggregates arising from mature copper/zinc superoxide dismutases resemble those observed in amyotrophic lateral sclerosis (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

amyotrophic lateral sclerosis

14 January 2020

main subject

0 references

0 references

4

1 reference

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14 January 2020

author name string

Young-Mi Hwang

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

Peter B Stathopulos

2

1 reference

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14 January 2020

Kristin Dimmick

3

1 reference

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14 January 2020

Hamid R Badiei

5

1 reference

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14 January 2020

Ming Sze Tong

6

1 reference

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14 January 2020

Jessica A O Rumfeldt

7

1 reference

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14 January 2020

Pu Chen

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

Vassili Karanassios

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

Elizabeth M Meiering

10

1 reference

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14 January 2020

language of work or name

English

0 references

publication date

25 October 2010

1 reference

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14 January 2020

number of pages

11

1 reference

Journal of Biological Chemistry

inferred from page(s)

published in

1 reference

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14 January 2020

volume

285

1 reference

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14 January 2020

issue

53

1 reference

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14 January 2020

page(s)

41701-41711

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

Disrupted Zinc-Binding Sites in Structures of Pathogenic SOD1 Variants D124V and H80R

14 January 2020

cites work

1 reference

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Mutation-dependent polymorphism of Cu,Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis

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Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form

6 July 2018

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A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop

6 July 2018

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Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.

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Functional features cause misfolding of the ALS-provoking enzyme SOD1

6 July 2018

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Experimental characterization of disordered and ordered aggregates populated during the process of amyloid fibril formation

6 July 2018

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Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS

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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

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Branching in amyloid fibril growth

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Amyloid formation by globular proteins under native conditions

6 July 2018

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Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme

6 July 2018

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Protein aggregation kinetics, mechanism, and curve-fitting: a review of the literature.

6 July 2018

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Initiation and elongation in fibrillation of ALS-linked superoxide dismutase

6 July 2018

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Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization

6 July 2018

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Structural classification of toxic amyloid oligomers

6 July 2018

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Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival

6 July 2018

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Systematic analysis of nucleation-dependent polymerization reveals new insights into the mechanism of amyloid self-assembly

6 July 2018

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

6 July 2018

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Structures of the G85R variant of SOD1 in familial amyotrophic lateral sclerosis

6 July 2018

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SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization

6 July 2018

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Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.

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Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences

6 July 2018

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Structural Characterization of Zinc-deficient Human Superoxide Dismutase and Implications for ALS

6 July 2018

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Metalation of the amyotrophic lateral sclerosis mutant glycine 37 to arginine superoxide dismutase (SOD1) apoprotein restores its structural and dynamical properties in solution to those of metalated wild-type SOD1.

6 July 2018

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Fiber-dependent amyloid formation as catalysis of an existing reaction pathway

6 July 2018

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6 July 2018

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

6 July 2018

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Folding versus aggregation: polypeptide conformations on competing pathways

6 July 2018

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

6 July 2018

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6 July 2018

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Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

6 July 2018

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Common dynamical signatures of familial amyotrophic lateral sclerosis-associated structurally diverse Cu, Zn superoxide dismutase mutants

6 July 2018

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A single mutation induces amyloid aggregation in the alpha-spectrin SH3 domain: analysis of the early stages of fibril formation

6 July 2018

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

6 July 2018

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

6 July 2018

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The rate and equilibrium constants for a multistep reaction sequence for the aggregation of superoxide dismutase in amyotrophic lateral sclerosis

6 July 2018

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Mechanism of prion propagation: amyloid growth occurs by monomer addition

6 July 2018

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Dimer destabilization in superoxide dismutase may result in disease-causing properties: structures of motor neuron disease mutants.

6 July 2018

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Protein measurement with the Folin phenol reagent

6 July 2018

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ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization

6 July 2018

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

6 July 2018

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

6 July 2018

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

6 July 2018

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Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS

6 July 2018

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Dynamic properties of the G93A mutant of copper-zinc superoxide dismutase as detected by NMR spectroscopy: implications for the pathology of familial amyotrophic lateral sclerosis

6 July 2018

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Do oxidatively modified proteins cause ALS?

6 July 2018

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

6 July 2018

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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.

6 July 2018

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Loss of in vitro metal ion binding specificity in mutant copper-zinc superoxide dismutases associated with familial amyotrophic lateral sclerosis

6 July 2018

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Hydrodynamic radii of native and denatured proteins measured by pulse field gradient NMR techniques.

6 July 2018

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Monitoring protein assembly using quasielastic light scattering spectroscopy

6 July 2018

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Designing conditions for in vitro formation of amyloid protofilaments and fibrils

6 July 2018

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6 July 2018

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Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins

6 July 2018

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6 July 2018

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

6 July 2018

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Kinetics of nucleation-controlled polymerization. A perturbation treatment for use with a secondary pathway

6 July 2018

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Total reconstitution of copper-zinc superoxide dismutase

6 July 2018

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6 July 2018

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Contribution of conformational stability and reversibility of unfolding to the increased thermostability of human and bovine superoxide dismutase mutated at free cysteines.

6 July 2018

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New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis

6 July 2018

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Atomic structures of wild-type and thermostable mutant recombinant human Cu,Zn superoxide dismutase

6 July 2018

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An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS.

6 July 2018

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

21 January 2018

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Fully metallated S134N Cu,Zn-superoxide dismutase displays abnormal mobility and intermolecular contacts in solution

21 January 2018

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

21 January 2018

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

21 January 2018

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Effect of environmental factors on the kinetics of insulin fibril formation: elucidation of the molecular mechanism

21 January 2018

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Analysis of protein aggregation kinetics.

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

21 January 2018

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Elucidation of the molecular mechanism during the early events in immunoglobulin light chain amyloid fibrillation. Evidence for an off-pathway oligomer at acidic pH.

21 January 2018

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Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

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

21 January 2018

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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

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Micro-heterogeneity and aggregation in beta2-microglobulin solutions: effects of temperature, pH, and conformational variant addition.

21 January 2018

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Structural transformations of oligomeric intermediates in the fibrillation of the immunoglobulin light chain LEN.

21 January 2018

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Partially folded intermediates as critical precursors of light chain amyloid fibrils and amorphous aggregates.

21 January 2018

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Sonication of proteins causes formation of aggregates that resemble amyloid

21 January 2018

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Huntingtin and mutant SOD1 form aggregate structures with distinct molecular properties in human cells

21 January 2018

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Transcriptional profiling in the lumbar spinal cord of a mouse model of amyotrophic lateral sclerosis: a role for wild-type superoxide dismutase 1 in sporadic disease?

21 January 2018

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Thioflavine T interaction with amyloid β-sheet structures

21 January 2018

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Equilibrium thermodynamic analysis of amyotrophic lateral sclerosis-associated mutant apo Cu,Zn superoxide dismutases

21 January 2018

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12 December 2020

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

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Amyotrophic lateral sclerosis-associated copper/zinc superoxide dismutase mutations preferentially reduce the repulsive charge of the proteins

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Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis

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12 December 2020

Cu/Zn superoxide dismutase-like immunoreactivity in Lewy body-like inclusions of sporadic amyotrophic lateral sclerosis

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Sporadic amyotrophic lateral sclerosis with dementia and Cu/Zn superoxide dismutase-positive Lewy body-like inclusions

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Immunohistochemical study on superoxide dismutases in spinal cords from autopsied patients with amyotrophic lateral sclerosis

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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

Structural studies of soluble oligomers of the Alzheimer beta-amyloid peptide

1 reference

12 December 2020

Formation of advanced glycation end-product-modified superoxide dismutase-1 (SOD1) is one of the mechanisms responsible for inclusions common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutation, and transgenic mice expressing

1 reference

12 December 2020

Involvement of the superoxide anion radical in the autoxidation of pyrogallol and a convenient assay for superoxide dismutase

1 reference

12 December 2020

UV spectroscopic studies of human erythrocyte superoxide dismutase

1 reference

12 December 2020

Swelling and vacuolisation of mitochondria in transgenic SOD1-ALS mice: a consequence of supranormal SOD1 expression?

1 reference

12 December 2020

Identifiers

DOI

10.1074/JBC.M110.113696

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20974846%20AND%20SRC:MED&resulttype=core&format=json

14 January 2020

1 reference