(Q34552535)

English

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

scientific article

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scholarly article

1 reference

Europe PubMed Central

3 August 2017

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

1 reference

Europe PubMed Central

3 August 2017

author name string

B A Amendt

1

1 reference

Europe PubMed Central

3 August 2017

W J Rhead

2

1 reference

Europe PubMed Central

3 August 2017

language of work or name

0 references

publication date

1 September 1985

1 reference

Europe PubMed Central

3 August 2017

Journal of Clinical Investigation

1 reference

Europe PubMed Central

3 August 2017

76

1 reference

Europe PubMed Central

3 August 2017

3

1 reference

Europe PubMed Central

3 August 2017

963-969

1 reference

Europe PubMed Central

3 August 2017

Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 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=423960

7 July 2018

Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 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=423960

7 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=423960

7 July 2018

Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

Vitamin-responsive inherited metabolic disorders

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

Acyl-CoA dehydrogenases and electron-transferring flavoprotein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

Dicarboxylic aciduria: The response to fasting

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

The syndrome of systemic carnitine deficiency: Clinical, morphologic, biochemical, and pathophysiologic features

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

Purification and characterization of a long-chain acyl-CoA hydrolase from rat liver microsomes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

7 July 2018

Dicarboxylic aciduria due to medium chain acyl CoA dehydrogenase defect. A cause of hypoglycemia in childhood.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

27 October 2018

Deficiency of medium chain fatty acylcoenzyme A dehydrogenase presenting as the sudden infant death syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=423960

27 October 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/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Acyl-CoA oxidase of rat liver: a new enzyme for fatty acid oxidation

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Profiles in altered metabolism. II--(omega -- 1)-hydroxyacid excretion in a case of episodic hypoglycemia

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Purification of the peroxisomal fatty acyl-CoA oxidase from rat liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

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/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The biological origin of ketotic dicarboxylic aciduria. In vivo and in vitro investigations of the omega-oxidation of C6-C16-monocarboxylic acids in unstarved, starved and diabetic rats

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

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/3840178

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/3840178

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/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The biological origin of ketotic dicarboxylic aciduria. II. In vivo and in vitro investigations of the beta-oxidation of C8-C16-dicarboxylic acids in unstarved, starved and diabetic rats

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

In vitro fibroblast studies in a patient with C6-C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Subunit structure of electron transfer flavoprotein

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

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/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Systemic carnitine deficiency simulating Reye syndrome

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

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/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Medium chain acyl-CoA dehydrogenase deficiency: apparent Km and Vmax values for fibroblast acyl-CoA dehydrogenase towards octanoyl CoA in patient and control cell lines

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The occurrence of adipic and suberic acid in urine from ketotic patients

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aliphatic C 6 -C 14 dicarboxylic acids in urine from an infant with fatal congenital lactic acidosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Urinary excretion of C6–C10 dicarboxylic acids in glycogen storage disease types I and III

1 reference

https://pubmed.ncbi.nlm.nih.gov/3840178

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1172/JCI112096

1 reference

Europe PubMed Central

3 August 2017

1 reference

Europe PubMed Central

3 August 2017

1 reference

Europe PubMed Central

3 August 2017

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