(Q45296637)

11 December 2017

Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A. (English)

1 reference

11 December 2017

Sven W Sauer

1

1 reference

11 December 2017

Jürgen G Okun

2

1 reference

11 December 2017

Marina A Schwab

3

1 reference

11 December 2017

Linda R Crnic

4

1 reference

11 December 2017

Georg F Hoffmann

5

1 reference

11 December 2017

Stephen I Goodman

6

1 reference

11 December 2017

David M Koeller

7

1 reference

11 December 2017

Stefan Kölker

8

1 reference

11 December 2017

language of work or name

English

0 references

publication date

19 April 2005

1 reference

Journal of Biological Chemistry

11 December 2017

1 reference

11 December 2017

280

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11 December 2017

21830-21836

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11 December 2017

23

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Neonatal screening for glutaryl-CoA dehydrogenase deficiency

11 December 2017

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Type I glutaric aciduria, part 1: natural history of 77 patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Outcome of the first 3-years of a DNA-based neonatal screening program for glutaric acidemia type 1 in Manitoba and northwestern Ontario, Canada

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Mutation analysis in glutaric aciduria type I.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Long-term follow-up, neurological outcome and survival rate in 28 Nordic patients with glutaric aciduria type 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric aciduria type I: outcome in the Republic of Ireland

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Pathomechanisms of neurodegeneration in glutaryl-CoA dehydrogenase deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Evidence that 3-hydroxyglutaric acid interacts with NMDA receptors in synaptic plasma membranes from cerebral cortex of young rats.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and β-Hydroxyglutarate: Explanation of the symptoms in glutaric aciduria?

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

3-Hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defenses in cerebral cortex of young rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Contribution of reactive oxygen species to 3-hydroxyglutarate neurotoxicity in primary neuronal cultures from chick embryo telencephalons

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

IGF-1 and bFGF reduce glutaric acid and 3-hydroxyglutaric acid toxicity in striatal cultures.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

3-Hydroxyglutaric acid fails to affect the viability of primary neuronal rat cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

On the neurotoxicity of glutaric, 3-hydroxyglutaric, and trans-glutaconic acids in glutaric acidemia type 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric aciduria I: creatine supplementation restores creatinephosphate levels in mixed cortex cells from rat incubated with 3-hydroxyglutarate

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric aciduria type I: pathomechanisms of neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Biochemical, pathologic and behavioral analysis of a mouse model of glutaric acidemia type I.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Three classes of inhibitors share a common binding domain in mitochondrial complex I (NADH:ubiquinone oxidoreductase).

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

The activation of aconitase by ferrous ions and reducing agents

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

An immunohistochemical study on alpha-ketoglutarate dehydrogenase complex in Parkinson's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Role of mitochondria in the etiology and pathogenesis of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Reduced Activities of Thiamine-Dependent Enzymes in the Brains and Peripheral Tissues of Patients With Alzheimer's Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Inhibition of the alpha-ketoglutarate dehydrogenase complex by the myeloperoxidase products, hypochlorous acid and mono-N-chloramine.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric acid induces oxidative stress in brain of young rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Potentiation of 3-hydroxyglutarate neurotoxicity following induction of astrocytic iNOS in neonatal rat hippocampal cultures

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Inhibition of α‐ketoglutarate dehydrogenase complex promotes cytochrome c release from mitochondria, caspase‐3 activation, and necrotic cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Recent progress on regulation of the mitochondrial permeability transition pore; a cyclosporin-sensitive pore in the inner mitochondrial membrane

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Overexpressed human mitochondrial thioredoxin confers resistance to oxidant-induced apoptosis in human osteosarcoma cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Thioredoxin-2 (TRX-2) is an essential gene regulating mitochondria-dependent apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Metabolic enzymes of mycobacteria linked to antioxidant defense by a thioredoxin-like protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Variations in spare electron transport chain capacity: The answer to an old riddle?

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Brain regional development of the activity of alpha-ketoglutarate dehydrogenase complex in the rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Case Report: Rhabdomyolysis in Glutaric Aciduria Type I

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric aciduria type 1: biochemical investigations and postmortem findings.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Glutaric aciduria: biochemical and morphologic considerations.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M502845200

21 January 2018

Identifiers

DOI

10.1074/JBC.M502845200

1 reference

11 December 2017

1 reference