(Q40409233)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

title

Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

amyotrophic lateral sclerosis

12 January 2020

main subject

0 references

1 reference

1

1 reference

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12 January 2020

author name string

Zuoshang Xu

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

Lawrence J Hayward

series ordinal

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

language of work or name

English

0 references

publication date

15 June 2005

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

12 January 2020

published in

1 reference

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12 January 2020

volume

280

1 reference

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12 January 2020

issue

33

1 reference

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12 January 2020

page(s)

29771-29779

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Amyotrophic lateral sclerosis

12 January 2020

cites work

1 reference

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21 January 2018

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

21 January 2018

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A fraction of yeast Cu,Zn-superoxide dismutase and its metallochaperone, CCS, localize to the intermembrane space of mitochondria. A physiological role for SOD1 in guarding against mitochondrial oxidative damage.

21 January 2018

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21 January 2018

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ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

21 January 2018

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

21 January 2018

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21 January 2018

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

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ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

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Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis

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

21 January 2018

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21 January 2018

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

21 January 2018

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Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.

21 January 2018

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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 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis

21 January 2018

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

21 January 2018

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Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase

21 January 2018

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21 January 2018

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Dynamic properties of the G93A mutant of copper-zinc superoxide dismutase as detected by NMR spectroscopy: implications for the pathology of familial amyotrophic lateral sclerosis

21 January 2018

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

21 January 2018

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Decreased metallation and activity in subsets of mutant superoxide dismutases associated with familial amyotrophic lateral sclerosis.

21 January 2018

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Familial amyotrophic lateral sclerosis-associated mutations decrease the thermal stability of distinctly metallated species of human copper/zinc superoxide dismutase

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

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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 January 2018

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21 January 2018

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

21 January 2018

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Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

21 January 2018

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21 January 2018

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21 January 2018

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Loss of in vitro metal ion binding specificity in mutant copper-zinc superoxide dismutases associated with familial amyotrophic lateral sclerosis

21 January 2018

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21 January 2018

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Exon 5 encoded domain is not required for the toxic function of mutant SOD1 but essential for the dismutase activity: identification and characterization of two new SOD1 mutations associated with familial amyotrophic lateral sclerosis

21 January 2018

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

21 January 2018

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Aggregation of ALS mutant superoxide dismutase expressed in Escherichia coli

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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An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis

21 January 2018

1 reference

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Amyotrophic lateral sclerosis: a proposed mechanism

21 January 2018

1 reference

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Inhibition of Chaperone Activity Is a Shared Property of Several Cu,Zn-Superoxide Dismutase Mutants That Cause Amyotrophic Lateral Sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M504039200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M504039200

21 January 2018

Identifiers

DOI

10.1074/JBC.M504039200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15958382%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

1 reference