(Q30159818)

8 June 2017

title

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

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8 June 2017

4

Julian P Whitelegge

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8 June 2017

Bryan Francis Shaw

1

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8 June 2017

Armando Durazo

2

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8 June 2017

Aram M Nersissian

3

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8 June 2017

Kym F Faull

5

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8 June 2017

Joan Selverstone Valentine

6

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8 June 2017

language of work or name

English

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publication date

26 April 2006

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Journal of Biological Chemistry

8 June 2017

published in

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8 June 2017

volume

281

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8 June 2017

issue

26

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8 June 2017

page(s)

18167-18176

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

8 June 2017

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

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

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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

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

21 January 2018

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

21 January 2018

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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

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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

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

21 January 2018

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

21 January 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

21 January 2018

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

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Prediction of the Absolute Aggregation Rates of Amyloidogenic Polypeptide Chains

21 January 2018

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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

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

21 January 2018

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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

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

21 January 2018

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

21 January 2018

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21 January 2018

Identifiers

DOI

10.1074/JBC.M600623200

1 reference

8 June 2017

1 reference