SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis (Q26785352)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

title

SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json

amyotrophic lateral sclerosis

24 February 2020

main subject

0 references

1 reference

3

1 reference

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24 February 2020

Dario Ronchi

2

1 reference

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24 February 2020

Giacomo P Comi

4

1 reference

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24 February 2020

Stefania Corti

5

1 reference

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24 February 2020

author name string

Francesco Tafuri

1

1 reference

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24 February 2020

language of work or name

English

0 references

publication date

25 August 2015

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json

Frontiers in Cellular Neuroscience

24 February 2020

published in

1 reference

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24 February 2020

volume

9

1 reference

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24 February 2020

page(s)

336

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A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

24 February 2020

cites work

1 reference

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Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

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How mitochondria produce reactive oxygen species

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SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization

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

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The copper chaperone for superoxide dismutase

7 April 2017

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State of play in amyotrophic lateral sclerosis genetics

7 April 2017

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Mechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosis

7 April 2017

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A disulfide relay system in the intermembrane space of mitochondria that mediates protein import

7 April 2017

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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.

7 April 2017

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

7 April 2017

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Exploring new pathways of neurodegeneration in ALS: the role of mitochondria quality control

7 April 2017

1 reference

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

7 April 2017

1 reference

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Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria

7 April 2017

1 reference

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Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS

7 April 2017

1 reference

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

7 April 2017

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ALS: a disease of motor neurons and their nonneuronal neighbors

7 April 2017

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Unraveling the mechanisms involved in motor neuron degeneration in ALS

7 April 2017

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Candidate genes for Parkinson disease: Lessons from pathogenesis

7 April 2017

1 reference

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Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.

28 September 2017

1 reference

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Mitochondrial dynamism and the pathogenesis of Amyotrophic Lateral Sclerosis.

28 September 2017

1 reference

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Therapeutic development in amyotrophic lateral sclerosis.

28 September 2017

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An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis

28 September 2017

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The mitochondrial disulfide relay system: roles in oxidative protein folding and beyond

28 September 2017

1 reference

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Defective mitochondrial dynamics is an early event in skeletal muscle of an amyotrophic lateral sclerosis mouse model

28 September 2017

1 reference

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Wild-type Cu/Zn superoxide dismutase stabilizes mutant variants by heterodimerization.

28 September 2017

1 reference

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Cellular therapy to target neuroinflammation in amyotrophic lateral sclerosis

28 September 2017

1 reference

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Small peptides against the mutant SOD1/Bcl-2 toxic mitochondrial complex restore mitochondrial function and cell viability in mutant SOD1-mediated ALS

28 September 2017

1 reference

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Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.

28 September 2017

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The changing scene of amyotrophic lateral sclerosis

28 September 2017

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Selective mitochondrial Ca2+ uptake deficit in disease endstage vulnerable motoneurons of the SOD1G93A mouse model of amyotrophic lateral sclerosis

28 September 2017

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Next-generation sequencing reveals DGUOK mutations in adult patients with mitochondrial DNA multiple deletions

28 September 2017

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Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS)

28 September 2017

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Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS.

28 September 2017

1 reference

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

28 September 2017

1 reference

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

28 September 2017

1 reference

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Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Mitochondria: the next (neurode)generation

28 September 2017

1 reference

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Copper depletion increases the mitochondrial-associated SOD1 in neuronal cells

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Mitochondrial respiratory chain dysfunction in muscle from patients with amyotrophic lateral sclerosis.

28 September 2017

1 reference

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ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2.

28 September 2017

1 reference

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Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space

28 September 2017

1 reference

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Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

28 September 2017

1 reference

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

28 September 2017

1 reference

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Oligomerization of mutant SOD1 in mitochondria of motoneuronal cells drives mitochondrial damage and cell toxicity

28 September 2017

1 reference

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Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

28 September 2017

1 reference

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Cu/Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of mitochondria and perturbs Ca2+ homeostasis in SOD1G93A mice

28 September 2017

1 reference

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Inflammatory cytokines increase mitochondrial damage in motoneuronal cells expressing mutant SOD1.

28 September 2017

1 reference

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Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

28 September 2017

1 reference

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

28 September 2017

1 reference

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Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences

28 September 2017

1 reference

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Cysteine 111 affects aggregation and cytotoxicity of mutant Cu,Zn-superoxide dismutase associated with familial amyotrophic lateral sclerosis

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Expression of a Cu,Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of neuroblastoma cells to infectious injury

28 September 2017

1 reference

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1.

28 September 2017

1 reference

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RCADiA: simple automation platform for comparative multidimensional protein identification technology

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Systematically perturbed folding patterns of amyotrophic lateral sclerosis (ALS)-associated SOD1 mutants

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

Toxic proteins in neurodegenerative disease

28 September 2017

1 reference

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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.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

31 May 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Cytochrome c oxidase subunit I microdeletion in a patient with motor neuron disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Amyotrophic lateral sclerosis linked to a novel SOD1 mutation with muscle mitochondrial dysfunction

21 January 2018

1 reference

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Factors controlling the uptake of yeast copper/zinc superoxide dismutase into mitochondria.

21 January 2018

1 reference

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Oxidation and reduction of cysteines in the intermembrane space of mitochondria: multiple facets of redox control

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

CHCHD10 mutations in Italian patients with sporadic amyotrophic lateral sclerosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Ultrastructural study of mitochondria in the spinal cord of transgenic mice with a G93A mutant SOD1 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Human copper chaperone for superoxide dismutase 1 mediates its own oxidation-dependent import into mitochondria.

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1.

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

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/10.3389%2FFNCEL.2015.00336

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Rapid disease progression correlates with instability of mutant SOD1 in familial ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

The mitochondrial disulfide relay system protein GFER is mutated in autosomal-recessive myopathy with cataract and combined respiratory-chain deficiency

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4548205

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

28 November 2018

1 reference

https://api.crossref.org/works/10.3389%2FFNCEL.2015.00336

Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1

21 January 2018

1 reference

12 December 2020

Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis

1 reference

12 December 2020

CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations

1 reference

12 December 2020

Deleterious role of superoxide dismutase in the mitochondrial intermembrane space

1 reference

12 December 2020

Identifiers

DOI

10.3389/FNCEL.2015.00336

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

1 reference

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json

24 February 2020

2 references

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26379505%20AND%20SRC:MED&resulttype=core&format=json