Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation. (Q45251257)

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scientific article published on 3 February 2005

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English	Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation.	scientific article published on 3 February 2005

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8 January 2020

Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation (English)

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15691826%20AND%20SRC:MED&resulttype=core&format=json

8 January 2020

amyotrophic lateral sclerosis

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Yoshiaki Furukawa

1

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8 January 2020

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Thomas V O'Halloran

2

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8 January 2020

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3 February 2005

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8 January 2020

Journal of Biological Chemistry

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8 January 2020

280

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8 January 2020

17

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8 January 2020

17266-17274

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8 January 2020

Unraveling the mechanisms involved in motor neuron degeneration in ALS

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

The copper chaperone for superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Undetectable intracellular free copper: the requirement of a copper chaperone for superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Mechanisms of biosynthesis of mammalian copper/zinc superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Aggregation of ALS mutant superoxide dismutase expressed in Escherichia coli

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Mechanism of Cu,Zn-superoxide dismutase activation by the human metallochaperone hCCS.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Conserved patterns in the Cu,Zn superoxide dismutase family.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis show enhanced formation of aggregates in vitro

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Thermodynamic and kinetic examination of protein stabilization by glycerol

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: decreased stability of the apo state

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

A comparative study of the relationship between protein structure and beta-aggregation in globular and intrinsically disordered proteins.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Protein folding and misfolding

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Sensitive enzyme immunoassay for human Cu/Zn superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Cellular distribution of superoxide dismutases in the rat CNS.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Redox environment of the cell as viewed through the redox state of the glutathione disulfide/glutathione couple

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Bicarbonate-dependent peroxidase activity of human Cu,Zn-superoxide dismutase induces covalent aggregation of protein: intermediacy of tryptophan-derived oxidation products

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Axonal transport of mutant superoxide dismutase 1 and focal axonal abnormalities in the proximal axons of transgenic mice.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Disulphide bridges in globular proteins

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Seeded conversion of recombinant prion protein to a disulfide-bonded oligomer by a reduction-oxidation process.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Cys10 mixed disulfides make transthyretin more amyloidogenic under mildly acidic conditions

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Toxic proteins in neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

A fraction of yeast Cu,Zn-superoxide dismutase and its metallochaperone, CCS, localize to the intermembrane space of mitochondria. A physiological role for SOD1 in guarding against mitochondrial oxidative damage.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Amyotrophic lateral sclerosis: a proposed mechanism

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Factors controlling the uptake of yeast copper/zinc superoxide dismutase into mitochondria.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Intrinsic differences in brain and spinal cord mitochondria: Implication for therapeutic interventions.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Mitochondria and apoptosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

ALS, SOD and peroxynitrite

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Substantial increase of protein stability by multiple disulphide bonds

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M500482200

21 January 2018

Identifiers

10.1074/JBC.M500482200

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15691826%20AND%20SRC:MED&resulttype=core&format=json

8 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15691826%20AND%20SRC:MED&resulttype=core&format=json

8 January 2020

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