Local unfolding in a destabilized, pathogenic variant of superoxide dismutase 1 observed with H/D exchange and mass spectrometry (Q30159818)

8 June 2017

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

1 reference

8 June 2017

4

Julian P Whitelegge

1 reference

8 June 2017

Bryan Francis Shaw

1

1 reference

8 June 2017

Armando Durazo

2

1 reference

8 June 2017

Aram M Nersissian

3

1 reference

8 June 2017

Kym F Faull

5

1 reference

8 June 2017

Joan Selverstone Valentine

6

1 reference

8 June 2017

language of work or name

English

0 references

publication date

26 April 2006

1 reference

Journal of Biological Chemistry

8 June 2017

1 reference

8 June 2017

281

1 reference

8 June 2017

26

1 reference

8 June 2017

18167-18176

1 reference

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

8 June 2017

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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/10.1074%2FJBC.M600623200

High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Loss of a metal-binding site in gelsolin leads to familial amyloidosis-Finnish type

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

A frequent ala 4 to val superoxide dismutase-1 mutation is associated with a rapidly progressive familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Myoglobin forms amyloid fibrils by association of unfolded polypeptide segments

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Identification and localization of slow, natural, cooperative unfolding in the hematopoietic cell kinase SH3 domain by amide hydrogen exchange and mass spectrometry.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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/10.1074%2FJBC.M600623200

Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

An obligatory intermediate controls the folding of the alpha-subunit of tryptophan synthase, a TIM barrel protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Hydrogen exchange properties of proteins in native and denatured states monitored by mass spectrometry and NMR.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Probing the non-covalent structure of proteins by amide hydrogen exchange and mass spectrometry

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Primary structure effects on peptide group hydrogen exchange

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Synthesis and characterization of a monomeric mutant Cu/Zn superoxide dismutase with partially reconstituted enzymic activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

The structure of holo and metal-deficient wild-type human Cu, Zn superoxide dismutase and its relevance to familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

EX1 hydrogen exchange and protein folding

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Mapping temperature-induced conformational changes in the Escherichia coli heat shock transcription factor sigma 32 by amide hydrogen exchange.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Protein misfolding and aggregation: new examples in medicine and biology of the dark side of the protein world

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Small-molecule-mediated stabilization of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants against unfolding and aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Reduced global cooperativity is a common feature underlying the amyloidogenicity of pathogenic lysozyme mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Local cooperativity in the unfolding of an amyloidogenic variant of human lysozyme

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Sequence and structural determinants of Cu, Zn superoxide dismutase aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Prediction of the Absolute Aggregation Rates of Amyloidogenic Polypeptide Chains

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Backbone Dynamics of Human Cu,Zn Superoxide Dismutase and of Its Monomeric F50E/G51E/E133Q Mutant: The Influence of Dimerization on Mobility and Function†

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Deuterium exchange mass spectrometry as a probe of protein kinase activation. Analysis of wild-type and constitutively active mutants of MAP kinase kinase-1

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

Amide hydrogen exchange determined by mass spectrometry: application to rabbit muscle aldolase

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M600623200

21 January 2018

Identifiers

DOI

10.1074/JBC.M600623200

1 reference

8 June 2017

1 reference