1.3.8.9: very-long-chain acyl-CoA dehydrogenase

This is an abbreviated version!
For detailed information about very-long-chain acyl-CoA dehydrogenase, go to the full flat file.

Reaction

Synonyms

ACADVL, acyl-CoA:(acceptor) 2,3-oxidoreductase, EC 1.3.99.3, MCAD, very long chain acyl-CoA dehydrogenase, very long-chain acyl-CoA dehydrogenase, very-long-chain acyl-CoA dehydrogenase, VLCAD, VLCADD

ECTree

     1 Oxidoreductases

         1.3 Acting on the CH-CH group of donors

             1.3.8 With a flavin as acceptor

                1.3.8.9 very-long-chain acyl-CoA dehydrogenase

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Results	in table
1	Activating Compound
15	AA Sequence
1	Application
3	Cloned(Commentary)
3	Cofactor
1	Crystallization (Commentary)
434	Disease/ Diagnostics
6	General Information
8	Localization
1	Molecular Weight [Da]
1	Natural Substrates/ Products (Substrates)
11	Organism
6	Pathway
5	PDB ID
1	Posttranslational Modification
2	Protein Variants
2	Purification (Commentary)
1	Reaction
11	Reference
11	Source Tissue
7	Substrates and Products (Substrate)
1	Subunits
25	Synonyms
1	Systematic Name

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Reference on EC 1.3.8.9 - very-long-chain acyl-CoA dehydrogenase

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REF.

AUTHORS

TITLE

JOURNAL

VOL.

PAGES

YEAR

ORGANISM (UNIPROT)

PUBMED ID

SOURCE

689339

Primassin, S.; Ter Veld, F.; Mayatepek, E.; Spiekerkoetter, U.

Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine

Pediatr. Res.

63

632-637

2008

Mus musculus

18317232

brenda

689340

Tajima, G.; Sakura, N.; Shirao, K.; Okada, S.; Tsumura, M.; Nishimura, Y.; Ono, H.; Hasegawa, Y.; Hata, I.; Naito, E.; Yamaguchi, S.; Shigematsu, Y.; Kobayashi, M.

Development of a new enzymatic diagnosis method for very-long-chain acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in Japan

Pediatr. Res.

64

667-672

2008

Homo sapiens

18670371

brenda

710755

Li, Z.; Zhai, Y.; Fang, J.; Zhou, Q.; Geng, Y.; Sun, F.

Purification, crystallization and preliminary crystallographic analysis of very-long-chain acyl-CoA dehydrogenase from Caenorhabditis elegans

Acta Crystallogr. Sect. F

66

426-430

2010

Caenorhabditis elegans

20383014

brenda

723908

Kabuyama, Y.; Suzuki, T.; Nakazawa, N.; Yamaki, J.; Homma, M.; Homma, Y.

Dysregulation of very long chain acyl-CoA dehydrogenase coupled with lipid peroxidation

Am. J. Physiol. Cell Physiol.

298

C107-C113

2010

Homo sapiens

19889959

brenda

739412

Zhang, Y.; Bharathi, S.S.; Rardin, M.J.; Uppala, R.; Verdin, E.; Gibson, B.W.; Goetzman, E.S.

SIRT3 and SIRT5 regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase

PLoS ONE

10

e0122297

2015

Homo sapiens (P49748)

25811481

brenda

741034

Isackson, P.; Sutton, K.; Hostetler, K.; Vladutiu, G.

Novel mutations in the gene encoding very long-chain acyl-CoA dehydrogenase identified in patients with partial carnitine palmitoyltransferase II deficiency

Muscle Nerve

47

224-229

2013

Homo sapiens (P49748)

23169530

brenda

741280

Cardoso, A.; Kakimoto, P.; Kowaltowski, A.

Diet-sensitive sources of reactive oxygen species in liver mitochondria: role of very long chain acyl-CoA dehydrogenases

PLoS ONE

8

e77088

2013

Mus musculus (P50544)

24116206

brenda

741392

Kakimoto, P.A.; Tamaki, F.K.; Cardoso, A.R.; Marana, S.R.; Kowaltowski, A.J.

H2O2 release from the very long chain acyl-CoA dehydrogenase

Redox Biol.

4

375-380

2015

Homo sapiens (P49748)

25728796

brenda

763263

Wang, Y.; Palmfeldt, J.; Gregersen, N.; Makhov, A.M.; Conway, J.F.; Wang, M.; McCalley, S.P.; Basu, S.; Alharbi, H.; St Croix, C.; Calderon, M.J.; Watkins, S.; Vockley, J.

Mitochondrial fatty acid oxidation and the electron transport chain comprise a multifunctional mitochondrial protein complex

J. Biol. Chem.

294

12380-12391

2019

Homo sapiens (P49748), Mus musculus (P50544)

31235473

brenda

763406

Hagemeijer, M.C.; Oussoren, E.; Ruijter, G.J.G.; Onkenhout, W.; Huidekoper, H.H.; Ebberink, M.S.; Waterham, H.R.; Ferdinandusse, S.; de Vries, M.C.; Huigen, M.C.D.G.; Kluijtmans, L.A.J.; Coene, K.L.M.; Blom, H.J.

Abnormal VLCADD newborn screening resembling MADD in four neonates with decreased riboflavin levels and VLCAD activity

JIMD Rep.

61

12-18

2021

Homo sapiens (P49748), Homo sapiens

34485012

brenda

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