(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

In more languages

default for all languages

No label defined

No description defined

Statements

scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

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

PubMed publication ID

3 August 2017

author name string

B A Amendt

1

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

W J Rhead

2

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

language of work or name

0 references

publication date

1 September 1985

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Journal of Clinical Investigation

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

76

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

3

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

963-969

1 reference

Europe PubMed Central

PubMed publication ID

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

PubMed publication ID

3 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Sitelinks

Wikipedia(0 entries)

Wikibooks(0 entries)

Wikinews(0 entries)

Wikiquote(0 entries)

Wikisource(0 entries)

Wikiversity(0 entries)

Wikivoyage(0 entries)

Wiktionary(0 entries)

Multilingual sites(0 entries)

Retrieved from "https://www.wikidata.org/w/index.php?title=Q34552535&oldid=1383816318"