Mitochondrial long chain fatty acid beta-oxidation in man and mouse (Q28246062)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19465148%20AND%20SRC:MED&resulttype=core&format=json

28 January 2020

title

Mitochondrial long chain fatty acid beta-oxidation in man and mouse (English)

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28 January 2020

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7

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28 January 2020

Sander M. Houten

11

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28 January 2020

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Ronald J A Wanders

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28 January 2020

author name string

Malika Chegary

1

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28 January 2020

Heleen te Brinke

2

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28 January 2020

Jos P N Ruiter

3

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28 January 2020

Frits A Wijburg

4

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Maria S K Stoll

5

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28 January 2020

Paul E Minkler

6

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Horst Schulz

8

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28 January 2020

Charles L Hoppel

9

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28 January 2020

language of work or name

English

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publication date

22 May 2009

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Biochimica et Biophysica Acta

28 January 2020

published in

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volume

1791

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issue

8

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28 January 2020

page(s)

806-815

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A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency

28 January 2020

cites work

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Disorders of mitochondrial fatty acyl-CoA beta-oxidation

20 March 2017

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Structural characterization of the mouse long-chain acyl-CoA dehydrogenase gene and 5' regulatory region

20 March 2017

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A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase

20 March 2017

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Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients

20 March 2017

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Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death

20 March 2017

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Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation

20 March 2017

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Assumption-free analysis of quantitative real-time polymerase chain reaction (PCR) data

20 March 2017

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Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy

7 April 2017

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Homozygous carnitine palmitoyltransferase 1b (muscle isoform) deficiency is lethal in the mouse

29 September 2017

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Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids

29 September 2017

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Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride

29 September 2017

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Fatty acid oxidation disorders

29 September 2017

1 reference

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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients

29 September 2017

1 reference

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Recognition and management of fatty acid oxidation defects: a series of 107 patients.

29 September 2017

1 reference

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Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death

29 September 2017

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Molecular basis of hepatic carnitine palmitoyltransferase I deficiency

29 September 2017

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Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors

29 September 2017

1 reference

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Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients

29 September 2017

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Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disorders.

2 June 2018

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Comparison of the binding affinities of acyl-CoA-binding protein and fatty-acid-binding protein for long-chain acyl-CoA esters

2 June 2018

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Quantification of carnitine and acylcarnitines in biological matrices by HPLC electrospray ionization-mass spectrometry

2 June 2018

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Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase.

27 November 2018

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Homozygous carnitine palmitoyltransferase 1a (liver isoform) deficiency is lethal in the mouse

27 November 2018

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Strategy for the isolation, derivatization, chromatographic separation, and detection of carnitine and acylcarnitines

27 November 2018

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Leaky beta-oxidation of a trans-fatty acid: incomplete beta-oxidation of elaidic acid is due to the accumulation of 5-trans-tetradecenoyl-CoA and its hydrolysis and conversion to 5-trans-tetradecenoylcarnitine in the matrix of rat mitochondria

27 November 2018

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Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress.

27 November 2018

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27 November 2018

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27 November 2018

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Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype

27 November 2018

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Assay of plasmalogens and polyunsaturated fatty acids (PUFA) in erythrocytes and fibroblasts.

27 November 2018

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An improved enzyme assay for carnitine palmitoyl transferase I in fibroblasts using tandem mass spectrometry

27 November 2018

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12 December 2020

Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids

1 reference

12 December 2020

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

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12 December 2020

A comparison of [9,10-3H]palmitic and [9,10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts

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12 December 2020

2,6-Dimethylheptanoyl-CoA is a specific substrate for long-chain acyl-CoA dehydrogenase (LCAD): evidence for a major role of LCAD in branched-chain fatty acid oxidation

1 reference

12 December 2020

An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion

1 reference

12 December 2020

Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Peroxisomes contain delta 3,5,delta 2,4-dienoyl-CoA isomerase and thus possess all enzymes required for the beta-oxidation of unsaturated fatty acids by a novel reductase-dependent pathway

1 reference

12 December 2020

Quantitative acylcarnitine profiling in fibroblasts using [U-13C] palmitic acid: an improved tool for the diagnosis of fatty acid oxidation defects

1 reference

12 December 2020

10.1016/J.BBALIP.2009.05.006

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