Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis. (Q44735743)

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English	Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis.	scientific article

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17 November 2019

Monomeric Cu,Zn-superoxide dismutase is a common misfolding intermediate in the oxidation models of sporadic and familial amyotrophic lateral sclerosis (English)

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17 November 2019

amyotrophic lateral sclerosis

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Avi Chakrabartty

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17 November 2019

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Neil R Cashman

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17 November 2019

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Rishi Rakhit

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17 November 2019

John P Crow

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17 November 2019

James R Lepock

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17 November 2019

Leslie H Kondejewski

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20 January 2004

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17 November 2019

Journal of Biological Chemistry

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17 November 2019

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17 November 2019

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17 November 2019

15499-15504

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17 November 2019

Genetic epidemiology of amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

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.M313295200

21 January 2018

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

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.M313295200

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.M313295200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

A receptor for advanced glycosylation endproducts (AGEs) is colocalized with neurofilament-bound AGEs and SOD1 in motoneurons of ALS: immunohistochemical study

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Sensitive enzyme immunoassay for human Cu/Zn superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

The molten globule state as a clue for understanding the folding and cooperativity of globular-protein structure.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

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.M313295200

21 January 2018

Determination and analysis of the 2 A-structure of copper, zinc superoxide dismutase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Copper(2+) binding to the surface residue cysteine 111 of His46Arg human copper-zinc superoxide dismutase, a familial amyotrophic lateral sclerosis mutant

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Solution structure of reduced monomeric Q133M2 copper, zinc superoxide dismutase (SOD). Why is SOD a dimeric enzyme?

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Role of the dimeric structure in Cu,Zn superoxide dismutase. pH-dependent, reversible denaturation of the monomeric enzyme from Escherichia coli

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Inhibiting transthyretin conformational changes that lead to amyloid fibril formation

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313295200

21 January 2018

Identifiers

10.1074/JBC.M313295200

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14734542%20AND%20SRC:MED&resulttype=core&format=json

17 November 2019

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14734542%20AND%20SRC:MED&resulttype=core&format=json

17 November 2019

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