Structural and biophysical properties of metal-free pathogenic SOD1 mutants A4V and G93A (Q27657645)

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9 February 2020

title

Structural and biophysical properties of metal-free pathogenic SOD1 mutants A4V and G93A (English)

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9 February 2020

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4

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9 February 2020

author name string

Ahmad Galaleldeen

1

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9 February 2020

Richard W Strange

2

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9 February 2020

Lisa J Whitson

3

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9 February 2020

Narendra Narayana

5

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9 February 2020

Alexander B Taylor

6

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9 February 2020

Jonathan P Schuermann

7

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9 February 2020

Stephen P Holloway

8

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9 February 2020

S Samar Hasnain

9

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9 February 2020

P John Hart

10

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9 February 2020

language of work or name

English

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publication date

1 October 2009

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9 February 2020

number of pages

8

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Archives of Biochemistry and Biophysics

inferred from page(s)

published in

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9 February 2020

volume

492

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issue

1-2

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9 February 2020

page(s)

40-47

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Functional features cause misfolding of the ALS-provoking enzyme SOD1

9 February 2020

cites work

1 reference

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

20 March 2017

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

20 March 2017

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

20 March 2017

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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

20 March 2017

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The copper chaperone for superoxide dismutase

20 March 2017

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Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase

20 March 2017

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Determination and analysis of the 2 A-structure of copper, zinc superoxide dismutase

20 March 2017

1 reference

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

20 March 2017

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Coot: model-building tools for molecular graphics

20 March 2017

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Refinement of macromolecular structures by the maximum-likelihood method

20 March 2017

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PHENIX: building new software for automated crystallographic structure determination

20 March 2017

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Improved methods for building protein models in electron density maps and the location of errors in these models

20 March 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

20 March 2017

1 reference

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Superoxide dismutase. An enzymic function for erythrocuprein (hemocuprein)

20 March 2017

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

20 March 2017

1 reference

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

20 March 2017

1 reference

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Assessment of phase accuracy by cross validation: the free R value. Methods and applications

7 April 2017

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Unraveling the mechanisms involved in motor neuron degeneration in ALS

7 April 2017

1 reference

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Recent developments in the PHENIX software for automated crystallographic structure determination

7 April 2017

1 reference

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

7 April 2017

1 reference

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

29 September 2017

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Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS.

29 September 2017

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

29 September 2017

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Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology

29 September 2017

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Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis.

29 September 2017

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Pathogenic superoxide dismutase structure, folding, aggregation and turnover

29 September 2017

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

29 September 2017

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

29 September 2017

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

29 September 2017

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

29 September 2017

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

29 September 2017

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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

29 September 2017

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Identification and interpretation of complexity in sedimentation velocity boundaries

29 September 2017

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

29 September 2017

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Superoxide dismutases. An adaptation to a paramagnetic gas.

29 September 2017

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

29 September 2017

1 reference

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Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis

2 June 2018

1 reference

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

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2787720

Faster superoxide dismutase mutants designed by enhancing electrostatic guidance

2 June 2018

1 reference

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

2 June 2018

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Variable Metallation of Human Superoxide Dismutase: Atomic Resolution Crystal Structures of Cu–Zn, Zn–Zn and As-isolated Wild-type Enzymes

27 November 2018

1 reference

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Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced form

27 November 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.

27 November 2018

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

27 November 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

27 November 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.

27 November 2018

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

27 November 2018

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

27 November 2018

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

27 November 2018

1 reference

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Electrostatic recognition between superoxide and copper, zinc superoxide dismutase

27 November 2018

1 reference

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Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models

27 November 2018

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

Analytical ultracentrifugation of complex macromolecular systems

1 reference

12 December 2020

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

1 reference

12 December 2020

10.1016/J.ABB.2009.09.020

Identifiers

DOI

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19800308%20AND%20SRC:MED&resulttype=core&format=json

9 February 2020

1 reference

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19800308%20AND%20SRC:MED&resulttype=core&format=json

9 February 2020

PubMed publication ID

19800308

2 references