Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis. (Q48302583)

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English	Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis.	scientific article

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20 March 2020

Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis (English)

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20 March 2020

amyotrophic lateral sclerosis

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Dominic J. Hare

5

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20 March 2020

Philip A. Doble

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20 March 2020

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20 March 2020

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20 March 2020

Anthony R. White

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20 March 2020

Peter J. Crouch

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20 March 2020

Blaine R Roberts

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Bradley J Turner

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Brett M Paterson

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Jeffrey R Liddell

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Alexandra Grubman

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20 March 2020

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Erin J McAllum

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20 March 2020

Kevin J Barnham

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Qiao-Xin Li

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20 March 2020

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Nastasia K H Lim

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20 March 2020

Paul S Donnelly

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20 March 2020

Katherine A Price

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20 March 2020

Sin Chun Lim

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20 March 2020

James L Hickey

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20 March 2020

Jared R Williams

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20 March 2020

Katja M Kanninen

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20 March 2020

Lin W Hung

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20 March 2020

Jean-Francois Monty

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20 March 2020

Roxana M Llanos

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20 March 2020

David R Kramer

20

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20 March 2020

Julian F B Mercer

21

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20 March 2020

Joseph S Beckman

26

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20 March 2020

Ashley I Bush

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20 March 2020

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1 June 2014

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20 March 2020

Journal of Neuroscience

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20 March 2020

34

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20 March 2020

23

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20 March 2020

8021-8031

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20 March 2020

describes a project that uses

1 reference

Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC6608261/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presymptomatic motor neuron loss and reactive astrocytosis in the SOD1 mouse model of amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CCS knockout mice establish an alternative source of copper for SOD in ALS

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetically Decreased Spinal Cord Copper Concentration Prolongs Life in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Oxidative damage to proteins in the spinal cord in amyotrophic lateral sclerosis (ALS).

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Kinetic stability of Cu/Zn superoxide dismutase is dependent on its metal ligands: implications for ALS.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Alteration of copper physiology in mice overexpressing the human Menkes protein ATP7A

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Overexpression of Abeta is associated with acceleration of onset of motor impairment and superoxide dismutase 1 aggregation in an amyotrophic lateral sclerosis mouse model

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pyrrolidine dithiocarbamate inhibits induction of immunoproteasome and decreases survival in a rat model of amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pyrrolidine dithiocarbamate activates Akt and improves spatial learning in APP/PS1 mice without affecting beta-amyloid burden

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Metalation of the amyotrophic lateral sclerosis mutant glycine 37 to arginine superoxide dismutase (SOD1) apoprotein restores its structural and dynamical properties in solution to those of metalated wild-type SOD1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ammonium tetrathiomolybdate delays onset, prolongs survival, and slows progression of disease in a mouse model for amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Metal-free superoxide dismutase-1 and three different amyotrophic lateral sclerosis variants share a similar partially unfolded beta-barrel at physiological temperature

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterization of a novel SOD-1(G93A) transgenic mouse line with very decelerated disease development

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Measuring copper and zinc superoxide dismutase from spinal cord tissue using electrospray mass spectrometry.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An impaired mitochondrial electron transport chain increases retention of the hypoxia imaging agent diacetylbis(4-methylthiosemicarbazonato)copperII.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Quantification strategies for elemental imaging of biological samples using laser ablation-inductively coupled plasma-mass spectrometry

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The hypoxia imaging agent CuII(atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Targeting of monomer/misfolded SOD1 as a therapeutic strategy for amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

High-resolution elemental bioimaging of Ca, Mn, Fe, Co, Cu, and Zn employing LA-ICP-MS and hydrogen reaction gas.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Therapeutic redistribution of metal ions to treat Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Using theoretical protein isotopic distributions to parse small-mass-difference post-translational modifications via mass spectrometry

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Therapeutic effects of CuII(atsm) in the SOD1-G37R mouse model of amyotrophic lateral sclerosis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Clinical trials of riluzole in patients with ALS. ALS/Riluzole Study Group-II.

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/24899723

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1523/JNEUROSCI.4196-13.2014

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24899723%20AND%20SRC:MED&resulttype=core&format=json

20 March 2020

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24899723%20AND%20SRC:MED&resulttype=core&format=json

20 March 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24899723%20AND%20SRC:MED&resulttype=core&format=json

20 March 2020

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