Aggregation of copper-zinc superoxide dismutase in familial and sporadic ALS (Q33742742)

29 July 2017

1 reference

title

Aggregation of copper-zinc superoxide dismutase in familial and sporadic ALS (English)

1 reference

amyotrophic lateral sclerosis

29 July 2017

main subject

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0 references

Madhuri Chattopadhyay

series ordinal

1

1 reference

29 July 2017

Joan Selverstone Valentine

series ordinal

2

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29 July 2017

publication date

1 July 2009

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Antioxidants & Redox Signaling

29 July 2017

published in

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29 July 2017

volume

11

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29 July 2017

issue

7

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29 July 2017

page(s)

1603-1614

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Initiation and elongation in fibrillation of ALS-linked superoxide dismutase

29 July 2017

cites work

1 reference

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Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis

21 June 2018

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

21 June 2018

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Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1.

21 June 2018

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

21 June 2018

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Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis.

21 June 2018

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Metal-free superoxide dismutase forms soluble oligomers under physiological conditions: a possible general mechanism for familial ALS

21 June 2018

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Binding of a single zinc ion to one subunit of copper-zinc superoxide dismutase apoprotein substantially influences the structure and stability of the entire homodimeric protein

21 June 2018

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Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants

21 June 2018

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How do ALS-associated mutations in superoxide dismutase 1 promote aggregation of the protein?

21 June 2018

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21 June 2018

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Onset and progression in inherited ALS determined by motor neurons and microglia

21 June 2018

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

21 June 2018

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

21 June 2018

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Systematically perturbed folding patterns of amyotrophic lateral sclerosis (ALS)-associated SOD1 mutants

21 June 2018

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Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

21 June 2018

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Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg.

21 June 2018

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

21 June 2018

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Unraveling the mechanisms involved in motor neuron degeneration in ALS

21 June 2018

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

21 June 2018

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

21 June 2018

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ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization

21 June 2018

1 reference

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Superoxide dismutase folding/unfolding pathway: role of the metal ions in modulating structural and dynamical features.

21 June 2018

1 reference

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

21 June 2018

1 reference

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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 June 2018

1 reference

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The perplexing role of copper-zinc superoxide dismutase in amyotrophic lateral sclerosis (Lou Gehrig's disease)

21 June 2018

1 reference

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

21 June 2018

1 reference

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

21 June 2018

1 reference

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New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: inclusions containing SOD1 in neurons and astrocytes.

21 June 2018

1 reference

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

21 June 2018

1 reference

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

21 June 2018

1 reference

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

21 June 2018

1 reference

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

21 June 2018

1 reference

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Thioflavine T interaction with synthetic Alzheimer's disease beta-amyloid peptides: detection of amyloid aggregation in solution

21 June 2018

1 reference

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

On the stability of bovine superoxide dismutase. The effects of metals.

21 June 2018

1 reference

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Superoxide dismutases. An adaptation to a paramagnetic gas.

21 June 2018

1 reference

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Contribution of conformational stability and reversibility of unfolding to the increased thermostability of human and bovine superoxide dismutase mutated at free cysteines.

21 June 2018

1 reference

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Sensitive enzyme immunoassay for human Cu/Zn superoxide dismutase.

21 June 2018

1 reference

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

Nuclear magnetic resonance and chemical modification studies of bovine erythrocyte superoxide dismutase: evidence for zinc-promoted organization of the active site structure

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

pH-dependent migration of copper(II) to the vacant zinc-binding site of zinc-free bovine erythrocyte superoxide dismutase

21 June 2018

1 reference

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

21 June 2018

1 reference

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

30 October 2018

1 reference

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Fully metallated S134N Cu,Zn-superoxide dismutase displays abnormal mobility and intermolecular contacts in solution

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

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

30 October 2018

1 reference

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Autocatalytic conversion of recombinant prion proteins displays a species barrier

30 October 2018

1 reference

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

Mechanisms of biosynthesis of mammalian copper/zinc superoxide dismutase

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2842589

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

30 October 2018

1 reference

12 December 2020

Ultrastructural study of aggregates in the spinal cord of transgenic mice with a G93A mutant SOD1 gene

1 reference

12 December 2020

Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice

1 reference

12 December 2020

Stabilization of mutant Cu/Zn superoxide dismutase (SOD1) protein by coexpressed wild SOD1 protein accelerates the disease progression in familial amyotrophic lateral sclerosis mice

1 reference

12 December 2020

Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice

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

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

1 reference

12 December 2020

Cu/Zn superoxide dismutase-like immunoreactivity in Lewy body-like inclusions of sporadic amyotrophic lateral sclerosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Sporadic amyotrophic lateral sclerosis with dementia and Cu/Zn superoxide dismutase-positive Lewy body-like inclusions

1 reference

12 December 2020

Identification of an apo-superoxide dismutase (Cu,Zn) pool in human lymphoblasts

1 reference

12 December 2020

Immunohistochemical study on superoxide dismutases in spinal cords from autopsied patients with amyotrophic lateral sclerosis

1 reference

12 December 2020

Identifiers

DOI

10.1089/ARS.2009.2536

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29 July 2017

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29 July 2017

1 reference