(Q46851121)

English

Many roads lead to Rome? Multiple modes of Cu,Zn superoxide dismutase destabilization, misfolding and aggregation in amyotrophic lateral sclerosis

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scholarly article

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Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

Many roads lead to Rome? Multiple modes of Cu,Zn superoxide dismutase destabilization, misfolding and aggregation in amyotrophic lateral sclerosis (English)

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

amyotrophic lateral sclerosis

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author name string

Helen R Broom

1

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

Jessica A O Rumfeldt

2

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

Elizabeth M Meiering

3

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

publication date

1 January 2014

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

Essays in Biochemistry

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

56

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

149-165

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

The changing scene of amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

The amyloid state of proteins in human diseases

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Transmissible proteins: expanding the prion heresy

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Early steps in oxidation-induced SOD1 misfolding: implications for non-amyloid protein aggregation in familial ALS.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

How do small single-domain proteins fold?

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Amyloid formation by globular proteins under native conditions

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

The Yin and Yang of protein folding

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Protein aggregation--pathways and influencing factors

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Energetics of oligomeric protein folding and association

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Conformational stability and folding mechanisms of dimeric proteins

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Metal-free ALS variants of dimeric human Cu,Zn-superoxide dismutase have enhanced populations of monomeric species

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Unfolding and folding kinetics of amyotrophic lateral sclerosis-associated mutant Cu,Zn superoxide dismutases

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Glutathionylation at Cys-111 induces dissociation of wild type and FALS mutant SOD1 dimers

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Initiation and elongation in fibrillation of ALS-linked superoxide dismutase

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Fibrillation precursor of superoxide dismutase 1 revealed by gradual tuning of the protein-folding equilibrium

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Disulfide scrambling describes the oligomer formation of superoxide dismutase (SOD1) proteins in the familial form of amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Sonication of proteins causes formation of aggregates that resemble amyloid

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Molecular dynamics using atomic-resolution structure reveal structural fluctuations that may lead to polymerization of human Cu Zn superoxide dismutase

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

ALS precursor finally shaken into fibrils

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Familial amyotrophic lateral sclerosis mutants of copper/zinc superoxide dismutase are susceptible to disulfide reduction

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Characterizing the role of Hsp90 in production of heat shock proteins in motor neurons reveals a suppressive effect of wild-type Hsf1.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Misfolded SOD1 and ALS: zeroing in on mitochondria

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

The transthyretin amyloidoses: from delineating the molecular mechanism of aggregation linked to pathology to a regulatory-agency-approved drug

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1042%2FBSE0560149

21 January 2018

Identifiers

10.1042/BSE0560149

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

25 December 2017

http://europepmc.org/abstract/MED/25131593

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