Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein (Q36727229)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

title

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

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

1 reference

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Marjatta Son

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Scot C Leary

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Nadine Romain

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Dennis R Winge

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Ronald G Haller

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

Jeffrey L Elliott

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

language of work or name

English

0 references

publication date

11 March 2008

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

4 January 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

volume

283

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

issue

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

page(s)

12267-12275

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

describes a project that uses

4 January 2020

ImageQuant

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC2431012/fullTextXML

inferred from PubMed Central ID database lookup

cites work

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Parkinson's disease brain mitochondrial complex I has oxidatively damaged subunits and is functionally impaired and misassembled.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Copper trafficking to the mitochondrion and assembly of copper metalloenzymes

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mitochondrial dysfunction and its role in motor neuron degeneration in ALS.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Defects in the biosynthesis of mitochondrial heme c and heme a in yeast and mammals

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Constitutive knockout of Surf1 is associated with high embryonic lethality, mitochondrial disease and cytochrome c oxidase deficiency in mice

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mammalian copper chaperone Cox17p has an essential role in activation of cytochrome C oxidase and embryonic development

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Defects in mitochondrial respiratory complexes III and IV, and human pathologies.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Cytochrome c oxidase and mitochondrial F1F0-ATPase (ATP synthase) activities in platelets and brain from patients with Alzheimer's disease

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Cytochrome c oxidase deficiency

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Involvement of mitochondrial ferredoxin and Cox15p in hydroxylation of heme O.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

A novel frameshift mutation of the mtDNA COIII gene leads to impaired assembly of cytochrome c oxidase in a patient affected by Leigh-like syndrome

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mutations in SCO2 are associated with a distinct form of hypertrophic cardiomyopathy and cytochrome c oxidase deficiency

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

A stop-codon mutation in the human mtDNA cytochrome c oxidase I gene disrupts the functional structure of complex IV.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Assembly of cytochrome-c oxidase in cultured human cells

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Aggregate formation in Cu,Zn superoxide dismutase-related proteins

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2431012

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

28 June 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

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/10.1074%2FJBC.M708523200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

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/10.1074%2FJBC.M708523200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Cytochrome c association with the inner mitochondrial membrane is impaired in the CNS of G93A-SOD1 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Motor neuron degeneration in amyotrophic lateral sclerosis mutant superoxide dismutase-1 transgenic mice: mechanisms of mitochondriopathy and cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Analysis of oxidative phosphorylation complexes in cultured human fibroblasts and amniocytes by blue-native-electrophoresis using mitoplasts isolated with the help of digitonin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Regulation of the heme A biosynthetic pathway in Saccharomyces cerevisiae

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Clinical, biochemical and molecular analyses of six patients with isolated cytochrome c oxidase deficiency due to mutations in the SCO2 gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Rett syndrome: clinical review and genetic update

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M708523200

Aerobic conditioning in patients with mitochondrial myopathies: physiological, biochemical, and genetic effects

21 January 2018

1 reference

12 December 2020

Early-onset multisystem mitochondrial disorder caused by a nonsense mutation in the mitochondrial DNA cytochrome C oxidase II gene

1 reference

12 December 2020

Disease progression in a transgenic model of familial amyotrophic lateral sclerosis is dependent on both neuronal and non-neuronal zinc binding proteins

1 reference

12 December 2020

Spinal muscular atrophy-like picture, cardiomyopathy, and cytochrome c oxidase deficiency

1 reference

12 December 2020

Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice

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

Native electrophoresis for isolation of mitochondrial oxidative phosphorylation protein complexes

1 reference

12 December 2020

Identifiers

DOI

10.1074/JBC.M708523200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json

4 January 2020

PubMed publication ID

18334481

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18334481%20AND%20SRC:MED&resulttype=core&format=json