Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. (Q33979028)

30 July 2017

Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. (English)

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amyotrophic lateral sclerosis

30 July 2017

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Jody B Proescher

1

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30 July 2017

Marjatta Son

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2

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30 July 2017

Jeffrey L Elliott

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3

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30 July 2017

Valeria C Culotta

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4

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30 July 2017

12 March 2008

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30 July 2017

Human Molecular Genetics

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30 July 2017

17

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30 July 2017

12

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30 July 2017

1728-1737

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describes a project that uses

30 July 2017

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC2900889/fullTextXML

inferred from PubMed Central ID database lookup

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5 July 2018

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5 July 2018

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

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

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

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21 January 2018

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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

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21 January 2018

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Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice

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12 December 2020

A gain of superoxide dismutase (SOD) activity obtained with CCS, the copper metallochaperone for SOD1

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12 December 2020

Copper activation of superoxide dismutase 1 (SOD1) in vivo. Role for protein-protein interactions with the copper chaperone for SOD1

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12 December 2020

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

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12 December 2020

Neuromuscular accumulation of mutant superoxide dismutase 1 aggregates in a transgenic mouse model of familial amyotrophic lateral sclerosis

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12 December 2020

10 Superoxide dismutase assays

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12 December 2020

Multiple protein domains contribute to the action of the copper chaperone for superoxide dismutase

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12 December 2020

Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation

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12 December 2020

Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA

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12 December 2020

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

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12 December 2020

Identifiers

DOI

10.1093/HMG/DDN063

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30 July 2017

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30 July 2017

1 reference