(Q37999478)

26 August 2017

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Misfolded SOD1 and ALS: zeroing in on mitochondria (English)

1 reference

amyotrophic lateral sclerosis

26 August 2017

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1 reference

Christine Vande Velde

series ordinal

2

object named as

Christine Vande Velde

1 reference

26 August 2017

Sarah Pickles

1

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26 August 2017

3 April 2012

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Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders

26 August 2017

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26 August 2017

13

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26 August 2017

4

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26 August 2017

333-340

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

26 August 2017

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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/10.3109%2F17482968.2012.648645

Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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/10.3109%2F17482968.2012.648645

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/10.3109%2F17482968.2012.648645

ALS-causing SOD1 mutations promote production of copper-deficient misfolded species

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Common dynamical signatures of familial amyotrophic lateral sclerosis-associated structurally diverse Cu, Zn superoxide dismutase mutants

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Unfolding and folding kinetics of amyotrophic lateral sclerosis-associated mutant Cu,Zn superoxide dismutases

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Denaturational stress induces formation of zinc-deficient monomers of Cu,Zn superoxide dismutase: implications for pathogenesis in amyotrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Molecular dynamics using atomic-resolution structure reveal structural fluctuations that may lead to polymerization of human Cu Zn superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Neurotoxic species of misfolded SOD1G93A recognized by antibodies against the P2X4 subunit of the ATP receptor accumulate in damaged neurons of transgenic animal models of amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Oxidative modification to cysteine sulfonic acid of Cys111 in human copper-zinc superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

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/10.3109%2F17482968.2012.648645

Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Mutant SOD1 from spinal cord of G93A rats is destabilized and binds to inner mitochondrial membrane

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Mitochondrial ubiquitin ligase MITOL ubiquitinates mutant SOD1 and attenuates mutant SOD1-induced reactive oxygen species generation

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

VDAC regulation by the Bcl-2 family of proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Bcl-2 family proteins regulate the release of apoptogenic cytochrome c by the mitochondrial channel VDAC

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Voltage-dependent anion channel 1-based peptides interact with Bcl-2 to prevent antiapoptotic activity

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Metabolic and functional differences between brain and spinal cord mitochondria underlie different predisposition to pathology

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Increased affinity for copper mediated by cysteine 111 in forms of mutant superoxide dismutase 1 linked to amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: mechanistic relationship and differential sensitivity to intervention

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F17482968.2012.648645

10.3109/17482968.2012.648645

21 January 2018

Identifiers

DOI

1 reference

26 August 2017

1 reference

PubMed ID

22471903