(Q35162951)

6 August 2017

title

Biochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G) (English)

1 reference

6 August 2017

Marjatta Son

1

1 reference

6 August 2017

Uma Srikanth

2

1 reference

6 August 2017

Krishna Puttaparthi

3

1 reference

6 August 2017

Christina Luther

4

1 reference

6 August 2017

Jeffrey L Elliott

5

1 reference

6 August 2017

language of work or name

English

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publication date

21 July 2011

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6 August 2017

number of pages

11

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Journal of Neurochemistry

inferred from page(s)

published in

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6 August 2017

volume

118

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6 August 2017

issue

5

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6 August 2017

page(s)

891-901

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Cu,Zn-superoxide dismutase increases toxicity of mutant and zinc-deficient superoxide dismutase by enhancing protein stability.

6 August 2017

cites work

1 reference

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

13 July 2018

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Exposure of hydrophobic surfaces initiates aggregation of diverse ALS-causing superoxide dismutase-1 mutants

13 July 2018

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Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis

13 July 2018

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

13 July 2018

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

The disulfide relay of the intermembrane space of mitochondria: an oxygen-sensing system?

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

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Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

The Erv1-Mia40 disulfide relay system in the intermembrane space of mitochondria

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Novel mutations that enhance or repress the aggregation potential of SOD1.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

A disulfide relay system in the intermembrane space of mitochondria that mediates protein import

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Mechanisms for activating Cu- and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

The copper chaperone for superoxide dismutase

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Three novel mutations and two variants in the gene for Cu/Zn superoxide dismutase in familial amyotrophic lateral sclerosis.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3155629

A vector for expressing foreign genes in the brains and hearts of transgenic mice

13 July 2018

2 references

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

21 January 2018

12 December 2020

Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

The ALS-associated mutation G93A in human copper-zinc superoxide dismutase selectively destabilizes the remote metal binding region

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

Aggregate formation in the spinal cord of mutant SOD1 transgenic mice is reversible and mediated by proteasomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

The disulfide relay system of mitochondria is required for the biogenesis of mitochondrial Ccs1 and Sod1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

?True? sporadic ALS associated with a novel SOD-1 mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2011.07360.X

Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1

21 January 2018

1 reference

12 December 2020

Disease progression in a transgenic model of familial amyotrophic lateral sclerosis is dependent on both neuronal and non-neuronal zinc binding proteins

1 reference

12 December 2020

Non-neuronal induction of immunoproteasome subunits in an ALS model: possible mediation by cytokines

1 reference

12 December 2020

Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA

1 reference

12 December 2020

10.1111/J.1471-4159.2011.07360.X

Identifiers

DOI

1 reference

6 August 2017

1 reference

6 August 2017

1 reference