(Q35808261)

English

The multiple acyl-coenzyme A dehydrogenation disorders, glutaric aciduria type II and ethylmalonic-adipic aciduria. Mitochondrial fatty acid oxidation, acyl-coenzyme A dehydrogenase, and electron transfer flavoprotein activities in fibroblasts

scientific article published on July 1986

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

The multiple acyl-coenzyme A dehydrogenation disorders, glutaric aciduria type II and ethylmalonic-adipic aciduria. Mitochondrial fatty acid oxidation, acyl-coenzyme A dehydrogenase, and electron transfer flavoprotein activities in fibroblasts (English)

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

0 references

dehydrogenation

1 reference

based on heuristic

inferred from title

Glutaric aciduria

1 reference

based on heuristic

inferred from title

author name string

B A Amendt

1

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

W J Rhead

2

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

language of work or name

0 references

publication date

1 July 1986

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

Journal of Clinical Investigation

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

78

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

1

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

205-213

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Multiple acyl-Co A dehydrogenation deficiency (MADD) in a boy with nonketotic hypoglycemia, hepatomegaly, muscle hypotonia and cardiomyopathy. Detection of N-isovalerylglutamic acid and its monoamide.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

The effect of tetrahydrofolate on the reduction of electron transfer flavoprotein by sarcosine and dimethylglycine dehydrogenases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

The Natural History of the Inherited Methylmalonic Acidemias

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Electron-transfer flavoprotein-ubiquinone oxidoreductase from pig liver: purification and molecular, redox, and catalytic properties

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

The acyl-CoA dehydrogenation deficiencies. Recent advances in the enzymic characterization and understanding of the metabolic and pathophysiological disturbances in patients with acyl-CoA dehydrogenation deficiencies

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Reaction of electron-transfer flavoprotein with electron-transfer flavoprotein-ubiquinone oxidoreductase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Deficiency of electron transfer flavoprotein or electron transfer flavoprotein:ubiquinone oxidoreductase in glutaric acidemia type II fibroblasts

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Recurrent Hypoglycemia Associated with Glutaric Aciduria Type II in an Adult

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Ethylmalonic-adipic aciduria. In vivo and in vitro studies indicating deficiency of activities of multiple acyl-CoA dehydrogenases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

11 July 2018

Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two newborn brothers. II. Morphology and pathogenesis.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

25 September 2018

Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two brothers. I. Clinical, metabolical, and biochemical findings.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=329551

25 September 2018

The purification and some properties of electron transfer flavoprotein and general fatty acyl coenzyme A dehydrogenase from pig liver mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Riboflavin-responsive ethylmalonic-adipic aciduria

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria type II: Report on a previously undescribed metabolic disorder

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Biochemical studies in a patient with defects in the metabolism of acyl‐CoA and sarcosine: Another possible case of glutaric aciduria type II

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Novel tritium release assays for isovaleryl-CoA and butyryl-CoA dehydrogenases

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria type II: in vitro studies on substrate oxidation, acyl-CoA dehydrogenases, and electron-transferring flavoprotein in cultured skin fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of general acyl CoA dehydrogenase by electron transfer flavoprotein semiquinone

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neonatal glutaric aciduria type II: an X-linked recessive inherited disorder

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Separation, properties, and regulation of acyl coenzyme A dehydrogenases from bovine heat and liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Purification and Properties of Rat Liver Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein1

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Induction of Acyl-CoA Dehydrogenases and Electron Transfer Flavoprotein and Their Roles in Fatty Acid Oxidation in Rat Liver Mitochondria1

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric acidemia type II: clinical, biochemical, and morphologic considerations

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Riboflavin deficiency and beta-oxidation systems in rat liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Purification and properties of electron-transferring flavoprotein from pig kidney

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dicarboxylic aciduria: deficient [1-14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The urinary excretion of ethylmalonic acid: What level requires further attention?

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Purification and characterization of 2-methyl-branched chain acyl coenzyme A dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Multiple acyl-CoA dehydrogenase deficiency occurring in pregnancy and caused by a defect in riboflavin metabolism in the mother. Study of a kindred with seven deaths in infancy: Value of riboflavin therapy in preventing this syndrome

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Congenital anomalies in glutaric aciduria type 2

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria type II: evidence for a defect related to the electron transfer flavoprotein or its dehydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria type II: biochemical investigation and treatment of a child diagnosed prenatally

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric acidaemia type II (multiple acyl-CoA dehydrogenation deficiency)

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaryl-CoA dehydrogenase activity determined with intact electron-transport chain: application to glutaric aciduria type II

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Electron-transferring flavoprotein deficiency in the multiple acyl-CoA dehydrogenation disorders, glutaric aciduria type II and ethylmalonic--adipic aciduria

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria type II: treatment with riboflavine, carnitine and insulin

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

An essential cysteine residue located in the vicinity of the FAD-binding site in short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glutaric aciduria; presence of glutaconic and beta-hydroxyglutaric acids in urine

1 reference

https://pubmed.ncbi.nlm.nih.gov/3722376

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1172/JCI112553

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

10 August 2017

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