(Q40100098)

17 September 2017

Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1. (English)

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amyotrophic lateral sclerosis

17 September 2017

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4

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17 September 2017

9

Gen Sobue

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17 September 2017

Jun-ichi Niwa

1

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17 September 2017

Shin-ichi Yamada

2

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17 September 2017

Shinsuke Ishigaki

3

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17 September 2017

Miho Takahashi

5

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17 September 2017

Masahisa Katsuno

6

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17 September 2017

Fumiaki Tanaka

7

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17 September 2017

Manabu Doyu

8

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17 September 2017

language of work or name

English

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publication date

31 July 2007

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Journal of Biological Chemistry

17 September 2017

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17 September 2017

282

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17 September 2017

38

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17 September 2017

28087-28095

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

17 September 2017

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

The role of arginine 143 in the electrostatics and mechanism of Cu,Zn superoxide dismutase: computational and experimental evaluation by mutational analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

The formation of protein disulphide bonds

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M704465200

Cellular distribution of superoxide dismutases in the rat CNS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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/10.1074%2FJBC.M704465200

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.M704465200

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.M704465200

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.M704465200

Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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.M704465200

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

NEDL1, a novel ubiquitin-protein isopeptide ligase for dishevelled-1, targets mutant superoxide dismutase-1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

A novel centrosomal ring-finger protein, dorfin, mediates ubiquitin ligase activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Comparative genomics of the RBR family, including the Parkinson's disease-related gene parkin and the genes of the ariadne subfamily

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Characterization of cytoplasmic alpha-synuclein aggregates. Fibril formation is tightly linked to the inclusion-forming process in cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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.M704465200

Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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.M704465200

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Evolution of CuZn superoxide dismutase and the Greek key beta-barrel structural motif

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Further characterization of human erythrocyte superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Accumulation of human SOD1 and ubiquitinated deposits in the spinal cord of SOD1G93A mice during motor neuron disease progression correlates with a decrease of proteasome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Proteasomal degradation of mutant superoxide dismutases linked to amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Structural properties and neuronal toxicity of amyotrophic lateral sclerosis-associated Cu/Zn superoxide dismutase 1 aggregates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

Degradation of Amyotrophic Lateral Sclerosis-linked Mutant Cu,Zn-Superoxide Dismutase Proteins by Macroautophagy and the Proteasome

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M704465200

21 January 2018

Identifiers

DOI

10.1074/JBC.M704465200

1 reference

17 September 2017

1 reference