(Q37018163)

17 August 2017

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

1 reference

17 August 2017

S. Mantagos

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A500826

7 July 2022

M. Genel

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A500826

7 July 2022

K. Tanaka

series ordinal

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A500826

7 July 2022

language of work or name

English

0 references

publication date

1 December 1979

1 reference

PMCID

371311

full work available at URL

17 August 2017

https://europepmc.org/articles/PMC371311

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A500826

https://europepmc.org/articles/PMC371311?pdf=render

7 July 2022

file format

Portable Document Format

online access status

open access

content deliverer

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A500826

Journal of Clinical Investigation

7 July 2022

published in

1 reference

17 August 2017

64

1 reference

17 August 2017

1580-1589

1 reference

17 August 2017

6

1 reference

Isovaleric acidemia: a new genetic defect of leucine metabolism

17 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Identification of ethylmalonic acid in urine of two patients with the vomitting sickness of Jamaica

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Investigation of the Mechanism of the Methylmalonyl-CoA Mutase Reaction with the Substrate Analogue: Ethylmalonyl-CoA

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Ketogenic diet containing medium-chain triglycerides

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Mammalian metabolism of glutaric acid

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Jamaican vomiting sickness. Biochemical investigation of two cases

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Metabolism in rats in vivo of RS-2-methylbutyrate and n-butyrate labeled with stable isotopes at various positions. Mechanism of biosynthesis and degradation of ethylmalonyl semialdehyde and ethylmalonic acid

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

ENZYMOLOGICAL DETERMINATION OF FREE CARNITINE CONCENTRATIONS IN RAT TISSUES

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Carnitine Deficiency of Human Skeletal Muscle with Associated Lipid Storage Myopathy: A New Syndrome

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=371311

Studies on omega-oxidation of fatty acids in vitro. I. Overall reaction and intermediate

4 September 2018

1 reference

12 December 2020

Specificity of carnitine action on fatty acid oxidation by heart muscle

1 reference

12 December 2020

New defect in fatty-acid metabolism with hypoglycemia and organic aciduria

1 reference

12 December 2020

Identification of n-hexanoylglycine in urines from two patients with Jamaican vomiting sickness

1 reference

12 December 2020

On the mode of action of hypoglycin A. 3. Isolation and identification of cis-4-decene-1,10-dioic, cis, cis-4,7-decadiene-1,10-dioic, cis-4-octene-1,8-dioic, glutaric, and adipic acids, N-(methylenecyclopropyl)acetylglycine, and N-isovalerylglycine

1 reference

12 December 2020

The Isolation and Identification of N-Isovalerylglycine from Urine of Patients with Isovaleric Acidemia

1 reference

12 December 2020

The Electron-transferring Flavoprotein as a Common Intermediate in the Mitochondrial Oxidation of Butyryl Coenzyme A and Sarcosine

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Glutaric aciduria; A “new” disorder of amino acid metabolism

1 reference

12 December 2020

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

1 reference

12 December 2020

Regulation of Gluconeogenesis by Fatty Acid Oxidation in Isolated Perfused Livers of Non-Starved Rats

1 reference

12 December 2020

SYSTEMS USED FOR THE TRANSPORT OF SUBSTRATES INTO MITOCHONDRIA

1 reference

12 December 2020

Identifiers

DOI

10.1172/JCI109619

1 reference

17 August 2017

1 reference

17 August 2017

1 reference

PMCID

371311